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The value of autopsy in pediatric cardiology and cardiovascular Surgery
The Value of Autopsy in Pediatric Cardiology and Cardiovascular Surgery CHEN-CHIH J. SUN, MD, GLADYS ALONSONZANA, MD, JON C. LOVE, MD, LING LI, MD, AND JOHN P. STRAUMANIS, MD Autopsy has been considered extremely helpful in determining the accuracy of the premorbid diagnosis. With recent advances in diagnostic imaging, the value of the autopsy has been called into question. The aim of this study was to assess the value of the pediatric autopsy in the current era of advanced diagnostic technology and sophisticated surgical intervention for congenital heart disease (CHD) and also to determine the autopsy’s potential impact on clinical management. We compared the data from the postmortem examination of 29 children with CHD with the data obtained before death and evaluated for the accuracy of the diagnosis of the cardiac anatomy as well as the cause of death. The diagnosis of the anatomy was confirmed in 13 cases (45%), additional cardiovascular abnor-
malities with potential clinical impact were found in 11 cases (38%), and additional abnormalities without clinical impact were found in 5 cases (17%). The cause of death was confirmed in 15 cases (52%), determined by autopsy in 12 cases (41%), and uncertain in 2 cases (7%). We conclude that the pediatric autopsy continues to provide clinically relevant information with a potential impact on patient management and to reveal unexpected causes of death in a significant proportion of patients with CHD. HUM PATHOL 34:491-496. © 2003 Elsevier Inc. All rights reserved. Key words: autopsy, congenital heart disease, pediatric, quality assurance. Abbreviations: CHD, congenital heart disease.
Autopsy has long been the gold standard for evaluating the accuracy of clinical diagnosis as well as determining the cause of death. Although there have been many studies detailing the benefits of autopsies in the adult population, pediatric autopsy data are surprisingly lacking.1 We chose to review our autopsy data from a group of children who had congenital heart disease (CHD) to determine the benefit of the postmortem examination in children with complex anatomy who underwent extensive premorbid examinations using both invasive and noninvasive procedures.
ers also assessed the clinical versus the autopsy cause of death and rated the findings as autopsy-confirmed clinical diagnosis (A), autopsy-determined cause of death (B), or cause of death remaining uncertain from all data reviewed (C). If there was disagreement among the 3 investigators as to the classification of the clinical impact or cause of death after initial review, then a consensus opinion was used.
MATERIALS AND METHODS The clinical and autopsy records of all patients with CHD who had a postmortem examination between November 1992 and October 1999 at the University of Maryland Hospital for Children were reviewed. The clinical data included, but were not limited to, physical examination, echocardiography, cardiac catheterization, and operative findings. These records were reviewed retrospectively by 3 of the investigators (C.C.J.S., J.L., and J.P.S.). The clinical data were reviewed and then the autopsy data were assessed according to its contribution to further clarifying the cardiovascular anatomy. In each case the value of the autopsy was classified as confirming the anatomy (Class A), finding additional aspects of the anatomy with potential impact on clinical management (Class B), or finding additional aspects of the anatomy without potential impact on clinical management (Class C). The same 3 review-
From the Departments of Pathology and Pediatrics, University of Maryland School of Medicine, Baltimore, MD. Accepted for publication December 2, 2002. Presented in part at the 90th annual meeting of the United States and Canadian Academy of Pathology, Atlanta, GA, March 2001. Address correspondence to Chen-Chih J. Sun, MD, Department of Pathology, University of Maryland Hospital, 22 South Greene Street, Baltimore, MD 21201. © 2003 Elsevier Inc. All rights reserved. 0046-8177/03/3405-0013$30.00/0 doi:10.1016/S0046-8177(03)000122-9
RESULTS Twenty-nine patients with CHD who had an autopsy between November 1992 and October 1999 were identified. There was an equal distribution of male (14) and female (15) patients, with a mean age of 1.4 years (range, 1 day to 16 years). All deaths except 1 occurred between November 1992 and December 1997. Either palliative or corrective cardiovascular surgery was performed on 23 patients. In this subgroup of patients, 12 died within 1 week of surgery, 3 died in the second to the fourth weeks after surgery, and 8 expired 1 month or longer after the surgery. The primary cardiac diagnosis, the classification of clinical and autopsy diagnosis correlation on cardiovascular anatomy, and agreement in cause of death rating for each patient are listed in Table 1. The cardiovascular anatomy was confirmed by autopsy (Class A) in 13 cases (45%). Additional cardiovascular abnormalities with the potential for clinical impact (Class B) were found on autopsy in 11 cases (38%), and additional abnormalities thought not have had an impact on clinical management (Class C) were found on autopsy in 5 cases (17%). More detailed clinical and autopsy data for Class B and Class C cases are given in Tables 2 and 3, respectively. The cause of death was confirmed by autopsy in 15 cases (52%) and determined by autopsy in 12 cases (41%). In 2 cases (7%), the cause of death remained uncertain after autopsy. In 1 of these cases (case 24), the autopsy was limited to the thoracic cavity only; in the other (case 21), the patient developed an
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TABLE 1. Primary Clinical Diagnosis and Correlation Classification for Cardiovascular Anatomy and Cause of Death Case No.
Clinical Diagnosis
1 2 3 4 5 6 7 8 9 10
Hypoplastic left ventricle Complete heart block associated with maternal connective tissue disease Pulmonary artery atresia with intact ventricular septum Pulmonary vein stenosis Complete atrioventricular canal defect Tetralogy of Fallot Atrial septal defect Hypertrophic cardiomyopathy, pulmonary hypertension Dilated cardiomyopathy, suspect myocarditis TAPVR to superior vena cava, pulmonary valve atresia with small right ventricle; atrioventricular canal defect; atrial septal defect TAPVR to portal vein; atrial septal defect; tricuspid valve regurgitation DiGeorge syndrome; preductal interrupted arch; ventricular septal defect; subaortic stenosis Tetralogy of Fallot Tetralogy of Fallot Atrioventricular canal defect with single atrioventricular valve; coarctation of aorta Transposition of the great arteries with ventricular inversion and hypoplastic right ventricle; coarctation of aorta Double-outlet right ventricle with hypoplastic left ventricle and abnormal systemic venous return Transposition of the great arteries with unbalanced atrioventricular canal defect and severe pulmonary valve stenosis Pulmonary valve atresia; abnormal tricuspid valve Complete atrioventricular canal defect Aortic valve stenosis; minimal mitral valve stenosis with dysplasia; atrial septal defect Transposition of the great arteries with double-outlet right ventricle Suspected CHD with normal heart by echocardiogram Tricuspid valve regurgitation; pulmonary valve incompetence; small left ventricle and aorta; severe mitral valve regurgitation Down’s syndrome, atrioventricular canal defect Polysplenia; dextrocardia; single ventricle; pulmonary valve stenosis; ventricular septal defect; atrial septal defect Hypoplastic left ventricle Pulmonary valve atresia with intact ventricular septum; tricuspid valve regurg.; right ventricle-right coronary artery fistula Coarcatation of aorta, left pulmonary artery sling
11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29
Cardiovascular Anatomy
Cause of Death
A A A A A A A A A A
A A A A A A A A A A
A A A B B B
A B B B B B
B B
B B
B B B B B B
B B C A A C
C C
A B
C C
A B
C
B
Abbreviation: TAPVR, total anomalous pulmonary venous return.
arrhythmia before death, but a cause of death was not identified by postmortem examination. Among the 12 cases in which the cause of death was identified at autopsy (Class B), causes of death included grade 3 pulmonary hypertension (case 12); lymphocytic laryngitis and pneumonitis (case 13); right ventricular infarct secondary to an abnormal right coronary artery ostium (case 14); bacterial endocarditis and sepsis (case 15); stenosis of a shunt or an anastomosis (cases 16 and 17); erroneous ligation of the left hepatic vein (case 18); acute left ventricular myocardial infarction secondary to an abnormal left coronary artery ostium (case 19); an unbalanced aortic valve canal defect with a small left ventricle, preductal stenosis of the aortic arch, and acute left ventricular infarct (case 20); cytomegalovirus infection (case 26); right ventricular necrosis (case 28); and infection and erosion of pulmonary artery banding and sepsis (case 29). Table 4 gives the clinical and autopsy diagnoses for these 12 cases with Class B correlation and 2 additional cases with Class C correlation for cause of death. Of the 23 patients who underwent surgery, 12 (52%) died of surgical complications. Eight patients died within 1 month after surgery secondary to stenosis
at anastomosis site (3 patients), erroneous ligation of a hepatic vein (1 patient), myocardial necrosis (3 patients), and coagulopathy after prolonged intraoperative hypotension (1 patient). Four patients died of late surgical complications more than 1 month after surgery. They included 1 case each of sepsis due to infection at the pulmonary artery banding site, ventricular septal defect patch injury to the aortic valve with aortic valve insufficiency, recurrent aortic arch stenosis, and thrombosis of a prosthetic mitral valve. Table 5 summarizes the surgical complications in these 12 postsurgical deaths. Other than cardiovascular lesions, infection was the most common cause of death, occurring in 8 patients (28%), including 1 case of cytomegalovirus, respiratory syncytial virus infection, and respiratory viral syndrome and 5 cases of bacterial pneumonia or sepsis. DISCUSSION In pediatric cardiology, autopsy has always been recognized as the ultimate study for establishing the definitive anatomical diagnosis and to determine the cause of death.2 It can serve as an additional source of
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TABLE 2. Clinical and Autopsy Diagnosis of Cases with Class B Correlation in the Cardiovascular System Case No.
Age
Clinical Diagnosis
14
10 months
15
14 days
16
12 days
17
20 days
18
7 months
19
2.5 years
20
48 days
Tetralogy of Fallot with infundibular stenosis; ventricular septal defect patch with residual defect (7 days); right ventricular dysfunction Atrioventricular canal defect with single atrioventricular valve; coarctation of aorta with small transverse aorta; aortic arch repair (8 days); atrioventricular canal patch and patch aortoplasty of transverse arch (⬍1 day) Transposition of the great arteries with ventricular inversion and hypoplastic right ventricle; aortic arch hypoplasia and coarctation; repair of coarctation, pulmonary artery banding, and ligation of patent ductus arteriosus (5 days); echocardiogram and cardiac catheterization (1 day): restrictive patent foramen ovale, hypoplastic mitral valve, double-outlet right ventricle; residual aortic arch stenosis; marked biventricular hypertrophy causing subaortic stenosis; anastomosis of main pulmonary artery to aorta, shunt from main pulmonary artery stump to reconstructed aorta and shunt from right pulmonary artery to right brachiocephalic artery (⬍1 day). Misaligned atrioventricular canal defect with hypoplastic left ventricle and moderate inlet ventricular septal defect; double-outlet right ventricle; subaortic stenosis; common atrium with small right superior vena cava; superior vena cava to left atrium; large patent ductus arteriosus, mild tricuspid valve regurgitation; interrupted inferior vena cava with azygous continuation; left hepatic vein to right atrium; stage 1 Norwood anastomosis of pulmonary artery to aorta and homograft (⬍1 day). Polysplenia syndrome; transposition of the great arteries with unbalanced atrioventricular canal defect and severe pulmonary valve stenosis; aorticopulmonary shunt (15 days); modified fenestrated Fontan procedure with ligation of Blalock-Taussig shunt and ligation of a presumed “hemiazygous” vein (2 days); postoperative atrioventricular valve regurgitation Pulmonary valve atresia; abnormal tricuspid valve; aortopulmonary artery shunt (2.5 years); modified Blalock-Taussig shunt (2 years); bidirectional Glenn shunt and right ventriculoplasty (10 months); left pulmonary artery balloon dilatation (2 months); modified Fontan (1 day); postoperative left ventricular dysfunction Complete atrioventricular canal defect, patch (⬍1 day); postoperative severe pulmonary hypertension and large residual ventricular septal defect, replacement of the patch (⬍1 day)
21
10 weeks
22
4 years
23 24
1 day 6 weeks
Discrepant Autopsy Diagnosis*
Atrial septal defect; aortic valve stenosis; minimal mitral valve stenosis with dysplasia; patent ductus arteriosus ligation (6 weeks); suspected coarctation of aorta; suspected left ventricular outflow obstruction or hypertrophic cardiomyopathy as cause of sudden arrhythmic death Transposition of the great arteries with double-outlet right ventricle; arterial-switch repair and ventricular septal defect closure (⬎3 years); postoperative severe atrial valve insufficiency; dilated left ventricle in echocardiogram, possible myocarditis Suspected CHD; normal heart by echocardiogram Tricuspid valve regurgitation; pulmonary valve incompetence; small left ventricle and aorta; severe mitral valve regurgitation; mitral valve replacement (26 days); questionable left. Circumflex artery patency; pulmonary hypertension
Dysplastic tricuspid valve; a “hood” above right coronary artery ostium; infarct of the right atrium and right ventricle Septal leaflet of the tricuspid valve fused to the septum; acute endocarditis of the right ventricle; grade 2 pulmonary hypertension arteriopathy Transposition of the great arteries with a right-sided morphological left ventricle connecting to the pulmonary artery; hypoplastic left-sided right ventricle to aorta, with aortic valve stenosis and subaortic stenosis; ventricular septal defect and status post atrial septectomy; dysplastic left atrioventricular valve; 1 mm patency at anastomosis site of shunt from main pulmonary artery stump to reconstructed aorta; 2 mm patency at shunt from right pulmonary artery to right brachiocephalic artery Dysplastic pulmonary valve; left hepatic vein to left atrium; supravalvular stenosis of pulmonary artery at anastomosis site of pulmonary artery and homograft
Complete atrioventricular canal defect; anterior atrioventricular valve attached to the papillary muscle of right ventricle and posterior atrioventricular valve attached to IV septum; ligation of left hepatic vein which drains into right atrium, not the new chamber; total anomalous pulmonary venous return to right atrium Angular origin of left coronary artery with shelf at aortic orifice
Small residual ventricular septal defect; unbalanced atrioventricular canal with small left ventricle, dilated, markedly hypertrophied right ventricle, and dilated right atrium; acute infarct of left ventricle; marked preductal stenosis of aortic isthmus Normal aortic valve; severe mitral valve stenosis with dysplasia; small left ventricle (1 cm) with marked hypertrophy; septal hypertrophy with mild subaortic stenosis; mild right ventricular hypertrophy Atrial valve cusp retracted and continuous with ventricular septal defect repair patch; single coronary ostium w/aortic wall ridge; dilated left ventricle; no myocarditis Ventricular septal defect Thrombosis of left coronary vein, with compression on left circumflex artery; grade 1 pulmonary hypertension
NOTE: The surgical procedure is in italics. The time shown in parentheses after a surgical procedure is the time interval between surgery and death. *Only a discrepant diagnosis in the cardiovascular system found at autopsy is listed. The major discrepant autopsy diagnosis is in bold.
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TABLE 3. Clinical and Autopsy Diagnosis of Cases With Class C Correlation in the Cardiovascular System Case No.
Age
Clinical Diagnosis
Discrepant Autopsy Diagnosis*
25
4 months
26
23 months
27
8 years
28
3 months
29
41 days
Down’s syndrome; atrioventricular canal defect; patch with small residual ventricular septal defect (25 days); atrioventricular disassociation Polysplenia; dextrocardia; single ventricle; pulmonary valve stenosis; ventricular septal defect and atrial septal defect; balloon pulmonary valvuloplasty (22.5 months); Blalock-Taussig shunt (22 months); poor ventricular function and patent shunt by catheter (4 days). Hypoplastic left ventricle; stage I Norwood (8 years); modified Glenn (6 years); Fontan procedure (2 years, 3 months); catheterization (2 months): LA outflow obstruction due to bulging baffle; hypoxemia, and pulmonary hypertension; repair of atrial septal baffle (1 day). Pulmonary valve atresia with intact ventricular septum; severe tricuspid valve regurgitation; coronary artery communicating to the right ventricular sinusoid; BlalockTaussig shunt (2 months); pulmonary valve valvotomy and patch angioplasty of torn main pulmonary artery (9 days); atrial septectomy (1 day); uncertain cause of continued right ventricular dysfunction Coarcatation of aorta; repair with pulmonary artery banding, and patent ductus arteriosus ligation (38 days); pulmonary artery banding erosion with hemorrhage, repair of pulmonary artery banding (⬍1 day).
Persistent left superior vena cava to coronary sinus Transposition of the great arteries with double outlet left ventricle and rudimentary right ventricle; atrioventricular canal defect with straddling tricuspid valve; atrial septal defect, ostium secundum type; right descending aorta Marked calcification of aortic conduit; grade 1 pulmonary hypertension
Pulmonary valve atresia with intact ventricular septum, mild tricuspid valve dysplasia; hypoplastic right ventricle with hypertrophy and necrosis of myocardium.
Bacterial infection and erosion of pulmonary artery band into the pulmonary artery
NOTE: The surgical procedure is in italics. The time shown in parentheses after a surgical procedure is the time interval between surgery and death. *Only a discrepant diagnosis in the cardiovascular system found at autopsy is listed. The major discrepant autopsy diagnosis is in bold.
information in evaluating the impact of new diagnostic techniques, monitoring the effects of treatment, and facilitating parental counseling.3 It has been argued that the availability of advanced diagnostic techniques might reduce the value of autopsy in this group of extensively studied patients. Such a claim is refuted by the present study. Although only 1 case in our series had a complete incorrect clinical diagnosis (case 23), the value of the autopsy is not limited to disclosing complete disagreement of clinical and autopsy diagnoses. In our series, 38% of cases had unsuspected cardiac abnormalities or surgical complications that, if known before death, might have changed clinical management and outcome. In 17% of cases, the additional cardiovascular findings, if known before death, would not have influenced the clinical management but still would have provided useful information to the clinicians. Knowledge of these findings may help improve future diagnostic accuracy. Through a better understanding of CHD and recent advances in diagnostic technique over the last decade, the clinical diagnosis is being reached before death in many cases. However, there is still a great need to establish the precise cause of death. As illustrated in this group of patients, the cause of death remained uncertain before autopsy despite the wide use of newer diagnostic techniques. Excluding surgical errors, an abnormality of the coronary artery ostium was the most common congenital cardiac anomaly that was not recognized before death, occurring in 3 (10%) of our patients (cases 14, 19, and 22). This is not surprising, because coronary artery anatomy can be difficult to discern. Pulmonary hypertension was recognized and
treated in all patients except 1 patient (case 12), who died with grade 3 pulmonary hypertensive arteriopathy that was not diagnosed clinically. Surgical complication was the most common cause of death in our patients (Table 5). All of the surgical complications were recognized before death except in 4 cases. There were 2 cases of anastomosis site stenosis (cases 16 and 17) and 1 case of pulmonary artery band erosion due to bacterial infection resulting in hemorrhage and sepsis (case 29). The fourth case (case 18) involved erroneous ligation of the left hepatic vein, which had been presumed to be a hemiazygous vein during a complicated surgery for a complex congenital heart disease. This surgical error, in conjunction with prolonged hypotension, resulted in hepatic necrosis, disseminated intravascular coagulopathy, and multifocal myocardial necrosis. The secondmost common cause of death in our series was infection, which was suspected clinically in all but 1 patient (case 26), who had poor ventricular function despite patent surgical shunts and died at home 4 days after discharge. Autopsy in this patient revealed systemic cytomegalovirus infection with myocarditis, pneumonitis, and hepatitis. The cause of death remained uncertain after autopsy in 2 patients, 1 of whom had an autopsy limited to the heart and lungs only, which limited the diagnostic value of the autopsy. We concur with Shana‘ah et al4 that a complete rather than a limited autopsy should be sought, because significant noncardiac causes of death may be found in these patients with complex congenital abnormalities. The results of our study were comparable to previous studies that compared autopsy and clinical diagnoses in children who had been treated medically or
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TABLE 4. Clinical and Autopsy Diagnosis of Cause of Death in Cases With Class B and Class C Correlation Case No.
Clinical Diagnosis
12
16
Respiratory failure secondary to recurrent aortic arch stenosis after modified Norwood procedure Tetralogy of Fallot with Blalock-Taussig shunt; died in emergency room of respiratory failure Adult respiratory distress syndrome; right ventricular dysfunction Died shortly after surgery for atrioventricular canal patch and patch aortoplasty of aortic arch; ?stenosis of aortic valve, mitral valve, or aortic arch Died during stage I Norwood procedure
17
Died during stage I Norwood procedure
18
Postoperative biventricular dysfunction
19
? Cause of left ventricular dysfunction
20
? Cause of postsurgical cardiac arrest
21
? Sudden arrhythmic death secondary to left ventricular outflow obstruction or hypertrophic cardiomyopathy Probable candida sepsis and cardiac failure Poor ventricular function of unknown etiology; died at home ? Cause of right ventricular dysfunction
13 14 15
24 26 28 29
Autopsy Diagnosis
Cause of Death Class
Grade 3 pulmonary hypertensive arteriopathy
B
Viral syndrome (lymphocytic laryngitis and pneumonitis)
B
Abnormal right coronary artery ostium with infarct of right ventricle and right atrium Bacterial endocarditis and sepsis, grade 2 hypertensive arteriopathy
B
Obstruction of right pulmonary artery to right brachiocephalic artery shunt and main pulmonary artery stump to reconstructed aorta shunt Pulmonary artery stenosis at anastomosis site to homograft Multifocal myocardial necrosis with fibrin thrombi in myocardial vessels, secondary to disseminated intravascular coagulation associated with hepatic necrosis/congestion due to erroneous ligation of left hepatic vein and prolonged postoperative hypotension Left ventricular infarct associated with abnormal left coronary artery ostium Acute left ventricular infarct secondary to unbalanced atrioventricular canal defect with small left ventricle (status post ventricular septal defect patch) and marked preductal stenosis of aortic arch ? Arrhythmia or mild subaortic stenosis secondary to septal hypertrophy Autopsy limited to heart and lungs Cytomegalovirus infection (pneumonitis, myocarditis, hepatitis) Myocardial necrosis of hypoplastic and hypertrophic right ventricle Bacterial infection of pulmonary artery band, acute mediastinitis and sepsis
Hemorrhage secondary to erosion and transection of pulmonary artery band; died during repair of pulmonary artery band
surgically for CHD. Russell and Berry2 reviewed the autopsies of 76 children who died between 1985 and 1987, and detected unsuspected abnormalities in 80% of cases and undiagnosed cardiac anomalies or surgical flaws in 38.2%, which contributed to death in 17.1% of cases. In a retrospective postmortem study of 59 patients who died between January 1992 and July 1995, Gatzoulis et al5 identified additional anatomical abnormalities in 13.6% of cases, and reported that the autopsy findings would probably have improved the outcome if they had been known before death in 8.5% of cases. In addition, the precise cause of death was con-
B
B B B
B B
C C B B B
firmed in 55.9% of cases, was clarified in 37.3%, and was uncertain in 7.8%. Shana’ah et al4 reviewed 56 autopsies performed from July 1993 through June 1996 and found significant clinically unexpected cardiovascular anomalies in 19.6% of cases and unsuspected acquired cardiovascular lesions in 28.6% of cases, which included thrombosis of the superior vena cava or prosthetic valve, lymphocytic myocarditis, and myocardial infarct. It has been reported that the frequency of major diagnostic errors in patients who died in the hospital was halved from 1972 to 1992, and this decline was
TABLE 5. Postsurgical Deaths With Surgical Complications No. of Deaths
Cause of Death (No. of Cases)
Early (ⱕ1 month)
8
Stenosis at anastomosis site (3) Erroneous ligation of hepatic vein (1) Myocardial necrosis due to prolonged intraoperative hypotension (3) Coagulopathy due to prolonged intraoperative hypotension (1)
Late (ⱖ1 month)
4
Sepsis due to infection at pulmonary artery banding site (1) Ventricular septal defect patch injury to aortic valve, causing insufficiency (1) Recurrent aortic arch stenosis (1) Thrombosis of prosthetic mitral valve (1)
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attributed to improved clinical skills and new diagnostic procedures.6 However, this did not appear to be the case in children with CHD, because autopsy continued to show a relatively high rate of unexpected findings. Several factors may explain this, including the use of new diagnostic techniques that alter the frequency of more definitive studies, such as cardiac catheterization, and the fact that more cardiovascular surgeries were performed in complex congenital heart diseases, such as hypoplastic left ventricle, on which corrective surgery would not have been attempted before. Accurate diagnosis is the cornerstone of excellence in clinical care. Our autopsies found lesions in the cardiovascular system that could have had impact on clinical management in 38% of cases and also identified unsuspected causes of death in 41% of cases. This frequency did not change between the mid-1980s and the late 1990s. Thus we conclude that autopsy continues to provide clinically useful information at a high
level in pediatric cardiology practice. To ensure topquality medical care, to improve future management, and to increase our understanding of CHD, autopsy should continue to be actively sought in all cases. REFERENCES 1. Stambouly JJ, Kahn E, Boxer RA: Correlation between clinical diagnoses and autopsy findings in critically ill children. Pediatrics 92:248-251, 1993 2. Russell GA, Berry PJ: Postmortem audit in a paediatric cardiology unit. J. Clin Pathol 42:912-918, 1989 3. Kay MH, Moodie DS, Sterba R: The value of the autopsy in congenital heart disease. Clin Pediatr (Phila) 30:450-454, 1991 4. Shanna’ah A Terk J, Schauer G: Post-mortem cause of death analysis in congenital heart disease. Mod Pathol 11:5[abstract 28], 1998 5. Gatzoulis MA, Sheppard MN, Ho SY: Value and impact of necropsy in paediatric cardiology. Heart 75:626-631, 1996 6. Sonderegger-Iseli K, Burger S, Muntwyler J: Diagnostic errors in three medical eras: A necropsy study. Lancet 355:2027-2031, 2000
496
malities with potential clinical impact were found in 11 cases (38%), and additional abnormalities without clinical impact were found in 5 cases (17%). The cause of death was confirmed in 15 cases (52%), determined by autopsy in 12 cases (41%), and uncertain in 2 cases (7%). We conclude that the pediatric autopsy continues to provide clinically relevant information with a potential impact on patient management and to reveal unexpected causes of death in a significant proportion of patients with CHD. HUM PATHOL 34:491-496. © 2003 Elsevier Inc. All rights reserved. Key words: autopsy, congenital heart disease, pediatric, quality assurance. Abbreviations: CHD, congenital heart disease.
Autopsy has long been the gold standard for evaluating the accuracy of clinical diagnosis as well as determining the cause of death. Although there have been many studies detailing the benefits of autopsies in the adult population, pediatric autopsy data are surprisingly lacking.1 We chose to review our autopsy data from a group of children who had congenital heart disease (CHD) to determine the benefit of the postmortem examination in children with complex anatomy who underwent extensive premorbid examinations using both invasive and noninvasive procedures.
ers also assessed the clinical versus the autopsy cause of death and rated the findings as autopsy-confirmed clinical diagnosis (A), autopsy-determined cause of death (B), or cause of death remaining uncertain from all data reviewed (C). If there was disagreement among the 3 investigators as to the classification of the clinical impact or cause of death after initial review, then a consensus opinion was used.
MATERIALS AND METHODS The clinical and autopsy records of all patients with CHD who had a postmortem examination between November 1992 and October 1999 at the University of Maryland Hospital for Children were reviewed. The clinical data included, but were not limited to, physical examination, echocardiography, cardiac catheterization, and operative findings. These records were reviewed retrospectively by 3 of the investigators (C.C.J.S., J.L., and J.P.S.). The clinical data were reviewed and then the autopsy data were assessed according to its contribution to further clarifying the cardiovascular anatomy. In each case the value of the autopsy was classified as confirming the anatomy (Class A), finding additional aspects of the anatomy with potential impact on clinical management (Class B), or finding additional aspects of the anatomy without potential impact on clinical management (Class C). The same 3 review-
From the Departments of Pathology and Pediatrics, University of Maryland School of Medicine, Baltimore, MD. Accepted for publication December 2, 2002. Presented in part at the 90th annual meeting of the United States and Canadian Academy of Pathology, Atlanta, GA, March 2001. Address correspondence to Chen-Chih J. Sun, MD, Department of Pathology, University of Maryland Hospital, 22 South Greene Street, Baltimore, MD 21201. © 2003 Elsevier Inc. All rights reserved. 0046-8177/03/3405-0013$30.00/0 doi:10.1016/S0046-8177(03)000122-9
RESULTS Twenty-nine patients with CHD who had an autopsy between November 1992 and October 1999 were identified. There was an equal distribution of male (14) and female (15) patients, with a mean age of 1.4 years (range, 1 day to 16 years). All deaths except 1 occurred between November 1992 and December 1997. Either palliative or corrective cardiovascular surgery was performed on 23 patients. In this subgroup of patients, 12 died within 1 week of surgery, 3 died in the second to the fourth weeks after surgery, and 8 expired 1 month or longer after the surgery. The primary cardiac diagnosis, the classification of clinical and autopsy diagnosis correlation on cardiovascular anatomy, and agreement in cause of death rating for each patient are listed in Table 1. The cardiovascular anatomy was confirmed by autopsy (Class A) in 13 cases (45%). Additional cardiovascular abnormalities with the potential for clinical impact (Class B) were found on autopsy in 11 cases (38%), and additional abnormalities thought not have had an impact on clinical management (Class C) were found on autopsy in 5 cases (17%). More detailed clinical and autopsy data for Class B and Class C cases are given in Tables 2 and 3, respectively. The cause of death was confirmed by autopsy in 15 cases (52%) and determined by autopsy in 12 cases (41%). In 2 cases (7%), the cause of death remained uncertain after autopsy. In 1 of these cases (case 24), the autopsy was limited to the thoracic cavity only; in the other (case 21), the patient developed an
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TABLE 1. Primary Clinical Diagnosis and Correlation Classification for Cardiovascular Anatomy and Cause of Death Case No.
Clinical Diagnosis
1 2 3 4 5 6 7 8 9 10
Hypoplastic left ventricle Complete heart block associated with maternal connective tissue disease Pulmonary artery atresia with intact ventricular septum Pulmonary vein stenosis Complete atrioventricular canal defect Tetralogy of Fallot Atrial septal defect Hypertrophic cardiomyopathy, pulmonary hypertension Dilated cardiomyopathy, suspect myocarditis TAPVR to superior vena cava, pulmonary valve atresia with small right ventricle; atrioventricular canal defect; atrial septal defect TAPVR to portal vein; atrial septal defect; tricuspid valve regurgitation DiGeorge syndrome; preductal interrupted arch; ventricular septal defect; subaortic stenosis Tetralogy of Fallot Tetralogy of Fallot Atrioventricular canal defect with single atrioventricular valve; coarctation of aorta Transposition of the great arteries with ventricular inversion and hypoplastic right ventricle; coarctation of aorta Double-outlet right ventricle with hypoplastic left ventricle and abnormal systemic venous return Transposition of the great arteries with unbalanced atrioventricular canal defect and severe pulmonary valve stenosis Pulmonary valve atresia; abnormal tricuspid valve Complete atrioventricular canal defect Aortic valve stenosis; minimal mitral valve stenosis with dysplasia; atrial septal defect Transposition of the great arteries with double-outlet right ventricle Suspected CHD with normal heart by echocardiogram Tricuspid valve regurgitation; pulmonary valve incompetence; small left ventricle and aorta; severe mitral valve regurgitation Down’s syndrome, atrioventricular canal defect Polysplenia; dextrocardia; single ventricle; pulmonary valve stenosis; ventricular septal defect; atrial septal defect Hypoplastic left ventricle Pulmonary valve atresia with intact ventricular septum; tricuspid valve regurg.; right ventricle-right coronary artery fistula Coarcatation of aorta, left pulmonary artery sling
11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29
Cardiovascular Anatomy
Cause of Death
A A A A A A A A A A
A A A A A A A A A A
A A A B B B
A B B B B B
B B
B B
B B B B B B
B B C A A C
C C
A B
C C
A B
C
B
Abbreviation: TAPVR, total anomalous pulmonary venous return.
arrhythmia before death, but a cause of death was not identified by postmortem examination. Among the 12 cases in which the cause of death was identified at autopsy (Class B), causes of death included grade 3 pulmonary hypertension (case 12); lymphocytic laryngitis and pneumonitis (case 13); right ventricular infarct secondary to an abnormal right coronary artery ostium (case 14); bacterial endocarditis and sepsis (case 15); stenosis of a shunt or an anastomosis (cases 16 and 17); erroneous ligation of the left hepatic vein (case 18); acute left ventricular myocardial infarction secondary to an abnormal left coronary artery ostium (case 19); an unbalanced aortic valve canal defect with a small left ventricle, preductal stenosis of the aortic arch, and acute left ventricular infarct (case 20); cytomegalovirus infection (case 26); right ventricular necrosis (case 28); and infection and erosion of pulmonary artery banding and sepsis (case 29). Table 4 gives the clinical and autopsy diagnoses for these 12 cases with Class B correlation and 2 additional cases with Class C correlation for cause of death. Of the 23 patients who underwent surgery, 12 (52%) died of surgical complications. Eight patients died within 1 month after surgery secondary to stenosis
at anastomosis site (3 patients), erroneous ligation of a hepatic vein (1 patient), myocardial necrosis (3 patients), and coagulopathy after prolonged intraoperative hypotension (1 patient). Four patients died of late surgical complications more than 1 month after surgery. They included 1 case each of sepsis due to infection at the pulmonary artery banding site, ventricular septal defect patch injury to the aortic valve with aortic valve insufficiency, recurrent aortic arch stenosis, and thrombosis of a prosthetic mitral valve. Table 5 summarizes the surgical complications in these 12 postsurgical deaths. Other than cardiovascular lesions, infection was the most common cause of death, occurring in 8 patients (28%), including 1 case of cytomegalovirus, respiratory syncytial virus infection, and respiratory viral syndrome and 5 cases of bacterial pneumonia or sepsis. DISCUSSION In pediatric cardiology, autopsy has always been recognized as the ultimate study for establishing the definitive anatomical diagnosis and to determine the cause of death.2 It can serve as an additional source of
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TABLE 2. Clinical and Autopsy Diagnosis of Cases with Class B Correlation in the Cardiovascular System Case No.
Age
Clinical Diagnosis
14
10 months
15
14 days
16
12 days
17
20 days
18
7 months
19
2.5 years
20
48 days
Tetralogy of Fallot with infundibular stenosis; ventricular septal defect patch with residual defect (7 days); right ventricular dysfunction Atrioventricular canal defect with single atrioventricular valve; coarctation of aorta with small transverse aorta; aortic arch repair (8 days); atrioventricular canal patch and patch aortoplasty of transverse arch (⬍1 day) Transposition of the great arteries with ventricular inversion and hypoplastic right ventricle; aortic arch hypoplasia and coarctation; repair of coarctation, pulmonary artery banding, and ligation of patent ductus arteriosus (5 days); echocardiogram and cardiac catheterization (1 day): restrictive patent foramen ovale, hypoplastic mitral valve, double-outlet right ventricle; residual aortic arch stenosis; marked biventricular hypertrophy causing subaortic stenosis; anastomosis of main pulmonary artery to aorta, shunt from main pulmonary artery stump to reconstructed aorta and shunt from right pulmonary artery to right brachiocephalic artery (⬍1 day). Misaligned atrioventricular canal defect with hypoplastic left ventricle and moderate inlet ventricular septal defect; double-outlet right ventricle; subaortic stenosis; common atrium with small right superior vena cava; superior vena cava to left atrium; large patent ductus arteriosus, mild tricuspid valve regurgitation; interrupted inferior vena cava with azygous continuation; left hepatic vein to right atrium; stage 1 Norwood anastomosis of pulmonary artery to aorta and homograft (⬍1 day). Polysplenia syndrome; transposition of the great arteries with unbalanced atrioventricular canal defect and severe pulmonary valve stenosis; aorticopulmonary shunt (15 days); modified fenestrated Fontan procedure with ligation of Blalock-Taussig shunt and ligation of a presumed “hemiazygous” vein (2 days); postoperative atrioventricular valve regurgitation Pulmonary valve atresia; abnormal tricuspid valve; aortopulmonary artery shunt (2.5 years); modified Blalock-Taussig shunt (2 years); bidirectional Glenn shunt and right ventriculoplasty (10 months); left pulmonary artery balloon dilatation (2 months); modified Fontan (1 day); postoperative left ventricular dysfunction Complete atrioventricular canal defect, patch (⬍1 day); postoperative severe pulmonary hypertension and large residual ventricular septal defect, replacement of the patch (⬍1 day)
21
10 weeks
22
4 years
23 24
1 day 6 weeks
Discrepant Autopsy Diagnosis*
Atrial septal defect; aortic valve stenosis; minimal mitral valve stenosis with dysplasia; patent ductus arteriosus ligation (6 weeks); suspected coarctation of aorta; suspected left ventricular outflow obstruction or hypertrophic cardiomyopathy as cause of sudden arrhythmic death Transposition of the great arteries with double-outlet right ventricle; arterial-switch repair and ventricular septal defect closure (⬎3 years); postoperative severe atrial valve insufficiency; dilated left ventricle in echocardiogram, possible myocarditis Suspected CHD; normal heart by echocardiogram Tricuspid valve regurgitation; pulmonary valve incompetence; small left ventricle and aorta; severe mitral valve regurgitation; mitral valve replacement (26 days); questionable left. Circumflex artery patency; pulmonary hypertension
Dysplastic tricuspid valve; a “hood” above right coronary artery ostium; infarct of the right atrium and right ventricle Septal leaflet of the tricuspid valve fused to the septum; acute endocarditis of the right ventricle; grade 2 pulmonary hypertension arteriopathy Transposition of the great arteries with a right-sided morphological left ventricle connecting to the pulmonary artery; hypoplastic left-sided right ventricle to aorta, with aortic valve stenosis and subaortic stenosis; ventricular septal defect and status post atrial septectomy; dysplastic left atrioventricular valve; 1 mm patency at anastomosis site of shunt from main pulmonary artery stump to reconstructed aorta; 2 mm patency at shunt from right pulmonary artery to right brachiocephalic artery Dysplastic pulmonary valve; left hepatic vein to left atrium; supravalvular stenosis of pulmonary artery at anastomosis site of pulmonary artery and homograft
Complete atrioventricular canal defect; anterior atrioventricular valve attached to the papillary muscle of right ventricle and posterior atrioventricular valve attached to IV septum; ligation of left hepatic vein which drains into right atrium, not the new chamber; total anomalous pulmonary venous return to right atrium Angular origin of left coronary artery with shelf at aortic orifice
Small residual ventricular septal defect; unbalanced atrioventricular canal with small left ventricle, dilated, markedly hypertrophied right ventricle, and dilated right atrium; acute infarct of left ventricle; marked preductal stenosis of aortic isthmus Normal aortic valve; severe mitral valve stenosis with dysplasia; small left ventricle (1 cm) with marked hypertrophy; septal hypertrophy with mild subaortic stenosis; mild right ventricular hypertrophy Atrial valve cusp retracted and continuous with ventricular septal defect repair patch; single coronary ostium w/aortic wall ridge; dilated left ventricle; no myocarditis Ventricular septal defect Thrombosis of left coronary vein, with compression on left circumflex artery; grade 1 pulmonary hypertension
NOTE: The surgical procedure is in italics. The time shown in parentheses after a surgical procedure is the time interval between surgery and death. *Only a discrepant diagnosis in the cardiovascular system found at autopsy is listed. The major discrepant autopsy diagnosis is in bold.
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TABLE 3. Clinical and Autopsy Diagnosis of Cases With Class C Correlation in the Cardiovascular System Case No.
Age
Clinical Diagnosis
Discrepant Autopsy Diagnosis*
25
4 months
26
23 months
27
8 years
28
3 months
29
41 days
Down’s syndrome; atrioventricular canal defect; patch with small residual ventricular septal defect (25 days); atrioventricular disassociation Polysplenia; dextrocardia; single ventricle; pulmonary valve stenosis; ventricular septal defect and atrial septal defect; balloon pulmonary valvuloplasty (22.5 months); Blalock-Taussig shunt (22 months); poor ventricular function and patent shunt by catheter (4 days). Hypoplastic left ventricle; stage I Norwood (8 years); modified Glenn (6 years); Fontan procedure (2 years, 3 months); catheterization (2 months): LA outflow obstruction due to bulging baffle; hypoxemia, and pulmonary hypertension; repair of atrial septal baffle (1 day). Pulmonary valve atresia with intact ventricular septum; severe tricuspid valve regurgitation; coronary artery communicating to the right ventricular sinusoid; BlalockTaussig shunt (2 months); pulmonary valve valvotomy and patch angioplasty of torn main pulmonary artery (9 days); atrial septectomy (1 day); uncertain cause of continued right ventricular dysfunction Coarcatation of aorta; repair with pulmonary artery banding, and patent ductus arteriosus ligation (38 days); pulmonary artery banding erosion with hemorrhage, repair of pulmonary artery banding (⬍1 day).
Persistent left superior vena cava to coronary sinus Transposition of the great arteries with double outlet left ventricle and rudimentary right ventricle; atrioventricular canal defect with straddling tricuspid valve; atrial septal defect, ostium secundum type; right descending aorta Marked calcification of aortic conduit; grade 1 pulmonary hypertension
Pulmonary valve atresia with intact ventricular septum, mild tricuspid valve dysplasia; hypoplastic right ventricle with hypertrophy and necrosis of myocardium.
Bacterial infection and erosion of pulmonary artery band into the pulmonary artery
NOTE: The surgical procedure is in italics. The time shown in parentheses after a surgical procedure is the time interval between surgery and death. *Only a discrepant diagnosis in the cardiovascular system found at autopsy is listed. The major discrepant autopsy diagnosis is in bold.
information in evaluating the impact of new diagnostic techniques, monitoring the effects of treatment, and facilitating parental counseling.3 It has been argued that the availability of advanced diagnostic techniques might reduce the value of autopsy in this group of extensively studied patients. Such a claim is refuted by the present study. Although only 1 case in our series had a complete incorrect clinical diagnosis (case 23), the value of the autopsy is not limited to disclosing complete disagreement of clinical and autopsy diagnoses. In our series, 38% of cases had unsuspected cardiac abnormalities or surgical complications that, if known before death, might have changed clinical management and outcome. In 17% of cases, the additional cardiovascular findings, if known before death, would not have influenced the clinical management but still would have provided useful information to the clinicians. Knowledge of these findings may help improve future diagnostic accuracy. Through a better understanding of CHD and recent advances in diagnostic technique over the last decade, the clinical diagnosis is being reached before death in many cases. However, there is still a great need to establish the precise cause of death. As illustrated in this group of patients, the cause of death remained uncertain before autopsy despite the wide use of newer diagnostic techniques. Excluding surgical errors, an abnormality of the coronary artery ostium was the most common congenital cardiac anomaly that was not recognized before death, occurring in 3 (10%) of our patients (cases 14, 19, and 22). This is not surprising, because coronary artery anatomy can be difficult to discern. Pulmonary hypertension was recognized and
treated in all patients except 1 patient (case 12), who died with grade 3 pulmonary hypertensive arteriopathy that was not diagnosed clinically. Surgical complication was the most common cause of death in our patients (Table 5). All of the surgical complications were recognized before death except in 4 cases. There were 2 cases of anastomosis site stenosis (cases 16 and 17) and 1 case of pulmonary artery band erosion due to bacterial infection resulting in hemorrhage and sepsis (case 29). The fourth case (case 18) involved erroneous ligation of the left hepatic vein, which had been presumed to be a hemiazygous vein during a complicated surgery for a complex congenital heart disease. This surgical error, in conjunction with prolonged hypotension, resulted in hepatic necrosis, disseminated intravascular coagulopathy, and multifocal myocardial necrosis. The secondmost common cause of death in our series was infection, which was suspected clinically in all but 1 patient (case 26), who had poor ventricular function despite patent surgical shunts and died at home 4 days after discharge. Autopsy in this patient revealed systemic cytomegalovirus infection with myocarditis, pneumonitis, and hepatitis. The cause of death remained uncertain after autopsy in 2 patients, 1 of whom had an autopsy limited to the heart and lungs only, which limited the diagnostic value of the autopsy. We concur with Shana‘ah et al4 that a complete rather than a limited autopsy should be sought, because significant noncardiac causes of death may be found in these patients with complex congenital abnormalities. The results of our study were comparable to previous studies that compared autopsy and clinical diagnoses in children who had been treated medically or
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TABLE 4. Clinical and Autopsy Diagnosis of Cause of Death in Cases With Class B and Class C Correlation Case No.
Clinical Diagnosis
12
16
Respiratory failure secondary to recurrent aortic arch stenosis after modified Norwood procedure Tetralogy of Fallot with Blalock-Taussig shunt; died in emergency room of respiratory failure Adult respiratory distress syndrome; right ventricular dysfunction Died shortly after surgery for atrioventricular canal patch and patch aortoplasty of aortic arch; ?stenosis of aortic valve, mitral valve, or aortic arch Died during stage I Norwood procedure
17
Died during stage I Norwood procedure
18
Postoperative biventricular dysfunction
19
? Cause of left ventricular dysfunction
20
? Cause of postsurgical cardiac arrest
21
? Sudden arrhythmic death secondary to left ventricular outflow obstruction or hypertrophic cardiomyopathy Probable candida sepsis and cardiac failure Poor ventricular function of unknown etiology; died at home ? Cause of right ventricular dysfunction
13 14 15
24 26 28 29
Autopsy Diagnosis
Cause of Death Class
Grade 3 pulmonary hypertensive arteriopathy
B
Viral syndrome (lymphocytic laryngitis and pneumonitis)
B
Abnormal right coronary artery ostium with infarct of right ventricle and right atrium Bacterial endocarditis and sepsis, grade 2 hypertensive arteriopathy
B
Obstruction of right pulmonary artery to right brachiocephalic artery shunt and main pulmonary artery stump to reconstructed aorta shunt Pulmonary artery stenosis at anastomosis site to homograft Multifocal myocardial necrosis with fibrin thrombi in myocardial vessels, secondary to disseminated intravascular coagulation associated with hepatic necrosis/congestion due to erroneous ligation of left hepatic vein and prolonged postoperative hypotension Left ventricular infarct associated with abnormal left coronary artery ostium Acute left ventricular infarct secondary to unbalanced atrioventricular canal defect with small left ventricle (status post ventricular septal defect patch) and marked preductal stenosis of aortic arch ? Arrhythmia or mild subaortic stenosis secondary to septal hypertrophy Autopsy limited to heart and lungs Cytomegalovirus infection (pneumonitis, myocarditis, hepatitis) Myocardial necrosis of hypoplastic and hypertrophic right ventricle Bacterial infection of pulmonary artery band, acute mediastinitis and sepsis
Hemorrhage secondary to erosion and transection of pulmonary artery band; died during repair of pulmonary artery band
surgically for CHD. Russell and Berry2 reviewed the autopsies of 76 children who died between 1985 and 1987, and detected unsuspected abnormalities in 80% of cases and undiagnosed cardiac anomalies or surgical flaws in 38.2%, which contributed to death in 17.1% of cases. In a retrospective postmortem study of 59 patients who died between January 1992 and July 1995, Gatzoulis et al5 identified additional anatomical abnormalities in 13.6% of cases, and reported that the autopsy findings would probably have improved the outcome if they had been known before death in 8.5% of cases. In addition, the precise cause of death was con-
B
B B B
B B
C C B B B
firmed in 55.9% of cases, was clarified in 37.3%, and was uncertain in 7.8%. Shana’ah et al4 reviewed 56 autopsies performed from July 1993 through June 1996 and found significant clinically unexpected cardiovascular anomalies in 19.6% of cases and unsuspected acquired cardiovascular lesions in 28.6% of cases, which included thrombosis of the superior vena cava or prosthetic valve, lymphocytic myocarditis, and myocardial infarct. It has been reported that the frequency of major diagnostic errors in patients who died in the hospital was halved from 1972 to 1992, and this decline was
TABLE 5. Postsurgical Deaths With Surgical Complications No. of Deaths
Cause of Death (No. of Cases)
Early (ⱕ1 month)
8
Stenosis at anastomosis site (3) Erroneous ligation of hepatic vein (1) Myocardial necrosis due to prolonged intraoperative hypotension (3) Coagulopathy due to prolonged intraoperative hypotension (1)
Late (ⱖ1 month)
4
Sepsis due to infection at pulmonary artery banding site (1) Ventricular septal defect patch injury to aortic valve, causing insufficiency (1) Recurrent aortic arch stenosis (1) Thrombosis of prosthetic mitral valve (1)
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attributed to improved clinical skills and new diagnostic procedures.6 However, this did not appear to be the case in children with CHD, because autopsy continued to show a relatively high rate of unexpected findings. Several factors may explain this, including the use of new diagnostic techniques that alter the frequency of more definitive studies, such as cardiac catheterization, and the fact that more cardiovascular surgeries were performed in complex congenital heart diseases, such as hypoplastic left ventricle, on which corrective surgery would not have been attempted before. Accurate diagnosis is the cornerstone of excellence in clinical care. Our autopsies found lesions in the cardiovascular system that could have had impact on clinical management in 38% of cases and also identified unsuspected causes of death in 41% of cases. This frequency did not change between the mid-1980s and the late 1990s. Thus we conclude that autopsy continues to provide clinically useful information at a high
level in pediatric cardiology practice. To ensure topquality medical care, to improve future management, and to increase our understanding of CHD, autopsy should continue to be actively sought in all cases. REFERENCES 1. Stambouly JJ, Kahn E, Boxer RA: Correlation between clinical diagnoses and autopsy findings in critically ill children. Pediatrics 92:248-251, 1993 2. Russell GA, Berry PJ: Postmortem audit in a paediatric cardiology unit. J. Clin Pathol 42:912-918, 1989 3. Kay MH, Moodie DS, Sterba R: The value of the autopsy in congenital heart disease. Clin Pediatr (Phila) 30:450-454, 1991 4. Shanna’ah A Terk J, Schauer G: Post-mortem cause of death analysis in congenital heart disease. Mod Pathol 11:5[abstract 28], 1998 5. Gatzoulis MA, Sheppard MN, Ho SY: Value and impact of necropsy in paediatric cardiology. Heart 75:626-631, 1996 6. Sonderegger-Iseli K, Burger S, Muntwyler J: Diagnostic errors in three medical eras: A necropsy study. Lancet 355:2027-2031, 2000
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