- Email: [email protected]
Hypertrophic cardiomyopathy and complete heart block in infancy
Volume 101 Number 6
arteries (TGA), ventricular septal defect (VSD), and subvalvular pulmonic stenosis. A left Blalock-Taussig shunt was performed at 13 years of age. At age 16 years, he complained of increiasing fatigue, cyanosis, and exertional chest pain. Following repeat cardiac catheterization, the proximal main PA and Blalock-Taussig shunt were ligated and the Rastelli procedure was performed; a Dacron patch was used to partially close the VSD and a 25mm Dacron conduit with a porcine aortic valve (Hancock Laboratories) were anastomosed to both the distal main PA and RV. Intraoperative pullback recordings revealed no gradient across the conduit or the left ventricular outflow tract. His postoperative course appeared uneventful. In 1977, 1 year later, repeat cardiac catheterization revealed an RV to PA peak systolic gradient of 55 mm Hg with a 20 mm Hg peak systolic gradient in the proximal conduit below the porcine valve, and a large ventricular left-to-right shunt. At reoperation, a repeat Rastelli procedure was carried out with closure of the VSD in a manner so that the left ventricle ejected into the aorta, and a new Dacron conduit was inserted. Recovery was uncomplicated and the patient remains asymptomatic; cardiac catheterization 1 year later in 1978 showed a peak systolic gradient of 20 mm Hg from the RV to PA with a 6 mm Hg intraconduit gradient and absent intracardiac shunting. The proximal and distal tubuIar portions of the removed original Dacron conduit contained a complete lining of friable pseudointima at least 3 mm thick (Fig. 1). This lining was adherent to the suture lines and at the RV anastomosis, the pannus was thickest and significantly narrowed the conduit orifice; the Hancock valve was normal. Histologically the portion of the pannus in contact with the Dacron conduit consisted mostly of collagen bundles while the luminal portion of the pannus consisted of organizing thrombus. The narrowest portion comprised polypoidal projections containing a myxoid matrix interspersed with stellate and smooth muscle cells (Fig. 2). The most frequent late complication following use of a Dacron conduit with a porcine aortic valve has been conduit obstruction documented to occur at three sites: the egress from the RV, the porcine valve, and the PA-to-conduit junction.1-d Man et aIs reported a case of thrombotic occlusion of a Hancock conduit following the Fontan procedure for tricuspid atresia. Our case illustrates another source of late conduit obstruction, that of pannus formation within the conduit. Thus patients with valved Dacron conduits should be closely followed even after early success, as in our patient. REFERENCES 1. Rocchini AP, Rosenthal A, Keane JF, Castaneda AR, Nadas AS: Hemodynamics after surgical repair with right ventricle to pulmonary artery conduit. Circulation 54:951, 1976. 2. Bailey WW, Kirklin JW, Bargeron LM Jr, Pacific0 AD, Kouchoukous NT: Late results with synthetic valved external conduits from venous ventricle to pulmonary arteries. Circulation 56(suppl 2):73, 1977. 3. Norwood WI, Freed MD, Rocchini AP, Bernhard WF, Castaneda AR: Experience with valved conduits for repair of congenital cardiac lesions. Ann Thorac Surg 24:223, 1977.
Brief
Communications
857
Clarkson S, Sade RM, Hohn A: Cliiical and hemodynamic results of extracardiac conduit reconstruction of the pulmonary artery. Clin Cardiol 3:42, 1980. 5. Mair DD, Fulton RE, Danielson GK: Thrombotic occlusion of Hancock conduit due to severe dehydration after Fontan operation. Mayo Clin Proc 53:397, 1978. 4.
Hypertrophic cardiomyopathy and complete heart block in infancy+ Barry J. Maron, M.D., Thomas M. Connor, M.D., Capt. (MC), USNR, and William C. Roberts, M.D. Bethesda, Md. Hypertrophic cardiomyopathy (HCM) is a congenital malformation that usually does not present clinically until adulthood.l, z However, HCM may be identified during the first year of life3m3 when it often manifests marked congestive heart failure (CHF).s While complete heart block (CHB) due to congenital conducting tissue disorde+ 7 may occur during the first few weeks of life, it is not usually associated with clinical deterioration during infancy.x. * This communication describes the unusual association of HCM and congenital CHB in an infant. A 2-month-old girl had weighed 2.8 kg at term birth by a noninsulin-dependent diabetic mother; CHB with heart rate 40 beats/minute (bpm) (Fig. 1) was known since the first day of life. Severe CHF developed by 3 days of age and an epicardial pacemaker was implanted at a ventricular rate of 100 bpm; digitalis and furosemide were begun. Cardiac catheterization showed left ventricular (LV) pressure 95/12 mm Hg without systolic gradient or mitral regurgitation; LV contractile pattern was normal with an ejection fraction of 0.70. CHF worsened, respiratory failure ensued, and her last month of life was further complicated by disseminated intravascular coagulation. Necropsy heart weight was 62 gm (normal 23 gm).l” The ventricular septum was disproportionately thickened relative to LV free walp’, ” (Fig. 2); septal thickness was 8 mm and LV free wall thickness was 3 mm (ratio 2.7). The coronary arteries were normal and there was no myocardial fibrosis or associated malformations. Marked disorganization of cardiac muscle cells was present in the ventricular septum (Fig. 3); longitudinal cut muscle fibers oriented at oblique and perpendicular angles to each other occupied 17% of. myocardium analyzed.13 LV free wall
From the Cardiology and Pathplogy Branches, Nationa Heart, Lung and Blood Institute, National Institutes of Health, and the Pediatric Cardiology Sectmn, Department of Pediatrics, National Naval Medical Center and United States Uniformed Health Services Medical School. Received
for publication
Feb. 20, 1981; accepted
Feb. 27, 1981.
Reprint requests: Barry J. Maron, M.D., Cardiology Bldg. 10, Room 7B-15, Bethesda, MD 20205.
Branch,
*The opinions and assertions expressed are those of the authors reflect the official position of the Navy.
0002-8703/81/060857
+ 04$00.40/0~1981
NHLBI, and do not
The C.V.Mosby
Co.
Fig. I. Rhythm strip showing rate of 120 bpm.
complete
atrioventricular
Fig. 2. Heart of the patient with hypertrophic cardiomyopathy and congenital complete heart blocky The ventricular septum (VS,Jis substantially thicker than the left ventricular free wall (LVJ Note the large left ventriccavity. ular papillary muscles. RV = right ventricular cardiac fibers were normally arranged. The AV node area was serially sectioned at 6 pm thickness and every tenth section was stained with hemotoxylin and eosin or elastic van Gieson. AV nodal tissue was normal; however, continuity of the conduction system was interrupted in the penetrating portion of the AV bundle (bundle of His) as it coursed through the central fibrous body (Fig. 4). Distal to this area of discontinuity the conducting tissue was normal and the AV bundle divided normally into left and right bundle branches. Because of necropsy findings, M-mode and two-dimensional echocardiograms were obtained in the infant’s parents. The mother’s echogram was normal but the father had asymmetric hypertrophy of the ventricular septum; septal thickness was 18 mm and LV free wall thickness was 10 mm (ratio 1.8). Congenital CHB most commonly occurs in structurally normal bearts, but 30% to 50% of such CHB is associated with a variety of congenital cardiovascular mafforma-
block with ventricular
rate of 40 bpm and atria1
tions.s-a Our patient demonstrates the previously undescribed association of congenital CHB and HCM CHB has been reported previously in a few patients with HCM1d-*l; however, since the age at which CHB was identified in those patients ranged from 10 to 71 years, it is unlikely that CHB was congenital. Our patient appears to have the typical genetically transmitted form of HCMZz: (1) disproportionate ventricular septal thickening,*‘, *’ (2) marked disorganization of cardiac muscle cells in the ventricular septum,lZ and (3) echocardiographic identification of asymmetric hypertrophy of the ventricular septum in the patient’s father.ZZ CHB in our patient appears to have emanated from a developmental defect in the conduction system-discontinuity in the AV bundle at the level of the central fibrous body. This anatomic abnormality of conducting tissue has not been reported in HCM,z5 but has been described m infants with congenital CHB and other congenital heart malformations.6 Whether the conduction system defect in our patient is a morphologic component of HCM or an etiologically independent abnormahty can not be determined with certainty at this time. The patient’s clinical course suggests that CHB and slow ventricular rate may not have contributed substantially to her clinical deterioration and eventual death. The patient’s CHF progressed markedly even after ventricular that our patient’s pacemaker insertion. The probability clinical deterioration was due primarily to the underlying cardiomyopathic process is supported by our previous presents with UZlF in experience that HClVl commonly the first year of life and leads to death in a substantial percentage of such patients5 This patient emphasizes the broad morphologic spectrum of HCM and suggests that HCM should be considered m an infant with CHB and CHF. REFERENCES
I.
2.
3.
4.
Frank S, Braunwald E: Idiopathic hypertrophic subaortic stenosis. Clinical analysis of 126 patients with emphasis on the natural history. Circulation 37:759, 1968. Shah PM, Adelman AG, Wigle ED, Gobel FL? Burchell HB, Hardarson T, Curie1 R, De la Calzada C, Qakley CM, Goodwin JF: The natural (and unnatural) course of hypertrophic obstructive cardiomyopathy. A multicenter study. Circ Res 34 and 35(suppl II):II-179, 1973. Barr PA, Celermajer JM, Bowdler JD, Cartmill TB: Idiopathic hypertrophic obstructive cardiomyopathy causing severe right ventricular outflow tract obstruction in infancy. Br Heart J 35:1109, 1973. Maron BJ, Edwards JE, Henry WL, Clark CE, Bingle GJ,
Volume 101 Number 6
Brief
Fig. 3. Area of ventricular septum showing disorganized arrangement of longitudinally cut cardi,ac muscle cells are oriented obliquely (Hematoxylin and eosin stain; original magnification x 100).
5.
6. 7.
8. 9.
10. 11.
12.
13.
14.
15
16.
Epstein SE: Asymmetric septal hypertrophy (ASH) in infancy. Circulation 50:809, 1974. Maron BJ, Tajik AJ, Ruttenberg HD, Graham TP, Atwood GF, Victorica BE, Lie JT, Roberts WC: Hypertrophic cardiomyopathy in infants: Clinical features and natural history. Circulation (In press) Lev M: Pathogenesis of congenital atrioventricular block. Progr Cardiovasc Dis 15:145, 1973. Jades TN, McKone RC, Hudspeth AS: De Subitaneis Mortibus X. Familial congenital heart block. Circulation 5i :379, 1975. Nakamura FF, Nadas AS: Complete heart block in infants and children. N Engl J Med 270:1261. 1964. Michai%son M, Eigle MA: Congeiital complete heart block: An international study of the natural history. Cardiovast Clin 4:86, 1972. Gould SE: Patlhology of the heart and blood vessels. 3rd ed. Springfield, Ill., 1968, Charles C Thomas, p 1139. Menges H, Brandenburg RO, Brown AL: The clinical, hemodynamic and pathologic diagnosis of muscular subvalvular aortic stenosis. Circulation 24:1126, 1961. Henry WL, Clark CE, Epstein SE: Asymmetric septal hypertrophy (ASH): Echocardiographic identification of the pathognomonic anatomic abnormality of IHSS. Circulation 47:225, 1973. Maron BJ, Satso N, Roberts WC, Edwards JE, Chandra RS: Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum: Comparison of fetuses and infants with and without congenital Reart disease and patients with hypertrophic cardiomyopathy. Circulation 60:685, 1979. Luisada AA: Subaortic muscular stenosis and complete heart block in an adolescent. Chicago Med School Q 25:169, 1965. Matlof HJ, Zener JC, Harrison DC: Idiopathic hypertrophic subaortic stenosis and heart block. Cycle-to-cycle variation as a functi& of alterations in preload and afterload. Am J Cardiol32:719? 1973. Johnson AD, Daily PO: Hypertrophic subaortic stenosis complicated by high degree heart block: Successful treatment with an atria1 synchronous ventricular pacemaker. Chest 67:491, llS75.
Conmunication~
959
of the cardiac muscle cells. Bundles and perpendicularly to each other.
Fig. 4. A section of the atrioventricular bundle area showing interruption in the penetrating portion of the bundle at the level of the central fibrous body. The two unconnected portions of conducting tissue are outlined by broken lines. VS = ventricular septum.
860 17.
18.
19.
20.
Brief
Communications
Spilkin S, Mitha AS, Matisonn RE, Chesler E: Complete heart block in a case of idiopathic hypertrophic subaortic stenosis. Noninvasive correlates with the timing of atria1 systole. Circulation 55:418, 1977. ChmieIewski CA, Riley RS? Mahendran A, Most AS: Complete beart block as a cause of syncope in asymmetric septal hypertrophy. AM HEART J 93:91, 1977. Tajik AJ, Guiliani ER, Frye RLY MeGoon DC, Nunn SL: Muscular subaortic stenosis witR complete heart biock. Am J Cardiol 31:101, 1973. Gavtilescu S, Gavrilescu M, Streian C, Luca C: Hypertrophic obstructive cardiomyopathy associated with complete heart block. Pathologic correlations in a case studied with His bundle electrography~ Acta Cardiol (Brux) 29:241,
1974. 21.
22.
23.
24.
25.
Gilgenkrantz JM, Cherrier F, Petitier H, Dodinot %, Houplan M, Legoux J: Cardiomyopathie obstructive du ventricule gauche avec bloc auriculo-ventriculaire complet; ConsideraGons thkrapeutiques. Arch Ma1 Coeur 61:4i9, 1968. Clark CE. Henrv WL. Erxtein SE: Familial mevalence and genetic tiansmi&ion ‘of -idiopathic hypertriphic subaortic stenosis. N Engl J Med 289:709, 1973. Maron BJ, Henry WL, Roberts WC, Epstein SE: Comparison of echocardiographic and necropsy measurements of ventricular wall thicknesses in patients with and without disproportionate septal thickening. Circulation 55~341, 1977. Henry WL, Ware J, Gardin Jl@, Hepner SI, &Kay J, Weiner M: Echocardiograpbic measurements in normal subjects. Growth-related changes that occur between infancy and early adulthood. Circulation 5?:278, 1978. James TN, Marshall TK: De Subitaneis Mortibus XII. Asymmetric hypertrophy of the heart. Circulation 51:1149, 19%.
Anomalous drainage to through the innominate syndrome
kft pulmonary venous the inferior wena cava and pericardiophrenic vein to the veinLeft-sided Scimitar
M. K. Mardini, M.D., N. A. Sakati, M~D.j and W. L. Nyhan, MOD.? PhD. Riyadh, Saudi Arabia7 and La Jolla, Calif. The term “Scimitar syndrome” was first used by Neil1 et a1.l to describe a disorder with a characteristic roentgenographic appearance in which there was anomalous pulmonary venous (PV) return from the right lung to the inferior vena cava (IVC) in a patient with a hypoplastic right lung and systemic arterial supply to the right lower lobe. The syndrome has since been considered a right-sided malformation. D’Cruz and Arcillaz reported an abnormal PV From the Department of Pediatrics at the King Faisa.1 Specialist Hospital and Resea~h Centus, Riyadh; and the Department of Pediatrics at the University of California San Diego. Reprink requests: Dr. M. K. Mardini, Dept. of Pediatrics, King F&al Speck&it Hospital and Research Centre, P.O. Box 3354, Riyadh, Saudi Arabia.
0002-8703/81/060860
+ 03$00.30/O
0 1981 The
c. V. Mosby
Co.
IV APV PCPV MS
lnnominafe Vein Anomalous Pulmonary Vein Pericardiophrenic Vein Moslem Sword
Fig. IA. Diagrammatic representation in the anterioposterior (A-P) view of anomalous venous connection from the left lung to the inferior vena cava (IVC), and via the pericardiophrenic vein to the innominate vein. The Moslem sword placed alongside provides a reminder of the similarity of its shape to that of the roentgenographic appearance of the anomalous pulmonary vein. SVC = SW perior vena cava; RA = right atrium; PA = pulmonary artery; A = aorta.
connection from a hypoplastic left lung to the IVC. It is the purpose of this report to present the second instance of this condition, and the first in which the anomalous left PV was found to drain into the IVC and into the innominate vein via the pericardiophrenic vein. A 2%-month-old Syrian male infant was referred for bronchoscopy because of a cough and pulmonary density. On examination he appeared well. X-rays showed hypoplasia or partial collapse of the left lung with hyperexpansion of the right, and herniation across the midline. A left-sided scimitar-shaped shadow was visualized in the left lower lung field crossing posterior to the heart and approaching the diaphragm at a 45-degree angle. Tomography was confirmatory. Lung scan with technicium-99 showed decreased perfusion of the left lung, more prominent in the left lower lung field. On ECG there was an RSR prime pattern on lead V, and transitional zone deviation to the left. Cardiac catheterization revealed a minimal step-up of oxygen saturation from the lower IVC to the right atrium and a slight increase in pulmonary artery (PA) pressure. PA angiogram showed a small hypoplastic left PA with abnormal distribution of blood to the left lung. The PV of the left lung joined together to
arteries (TGA), ventricular septal defect (VSD), and subvalvular pulmonic stenosis. A left Blalock-Taussig shunt was performed at 13 years of age. At age 16 years, he complained of increiasing fatigue, cyanosis, and exertional chest pain. Following repeat cardiac catheterization, the proximal main PA and Blalock-Taussig shunt were ligated and the Rastelli procedure was performed; a Dacron patch was used to partially close the VSD and a 25mm Dacron conduit with a porcine aortic valve (Hancock Laboratories) were anastomosed to both the distal main PA and RV. Intraoperative pullback recordings revealed no gradient across the conduit or the left ventricular outflow tract. His postoperative course appeared uneventful. In 1977, 1 year later, repeat cardiac catheterization revealed an RV to PA peak systolic gradient of 55 mm Hg with a 20 mm Hg peak systolic gradient in the proximal conduit below the porcine valve, and a large ventricular left-to-right shunt. At reoperation, a repeat Rastelli procedure was carried out with closure of the VSD in a manner so that the left ventricle ejected into the aorta, and a new Dacron conduit was inserted. Recovery was uncomplicated and the patient remains asymptomatic; cardiac catheterization 1 year later in 1978 showed a peak systolic gradient of 20 mm Hg from the RV to PA with a 6 mm Hg intraconduit gradient and absent intracardiac shunting. The proximal and distal tubuIar portions of the removed original Dacron conduit contained a complete lining of friable pseudointima at least 3 mm thick (Fig. 1). This lining was adherent to the suture lines and at the RV anastomosis, the pannus was thickest and significantly narrowed the conduit orifice; the Hancock valve was normal. Histologically the portion of the pannus in contact with the Dacron conduit consisted mostly of collagen bundles while the luminal portion of the pannus consisted of organizing thrombus. The narrowest portion comprised polypoidal projections containing a myxoid matrix interspersed with stellate and smooth muscle cells (Fig. 2). The most frequent late complication following use of a Dacron conduit with a porcine aortic valve has been conduit obstruction documented to occur at three sites: the egress from the RV, the porcine valve, and the PA-to-conduit junction.1-d Man et aIs reported a case of thrombotic occlusion of a Hancock conduit following the Fontan procedure for tricuspid atresia. Our case illustrates another source of late conduit obstruction, that of pannus formation within the conduit. Thus patients with valved Dacron conduits should be closely followed even after early success, as in our patient. REFERENCES 1. Rocchini AP, Rosenthal A, Keane JF, Castaneda AR, Nadas AS: Hemodynamics after surgical repair with right ventricle to pulmonary artery conduit. Circulation 54:951, 1976. 2. Bailey WW, Kirklin JW, Bargeron LM Jr, Pacific0 AD, Kouchoukous NT: Late results with synthetic valved external conduits from venous ventricle to pulmonary arteries. Circulation 56(suppl 2):73, 1977. 3. Norwood WI, Freed MD, Rocchini AP, Bernhard WF, Castaneda AR: Experience with valved conduits for repair of congenital cardiac lesions. Ann Thorac Surg 24:223, 1977.
Brief
Communications
857
Clarkson S, Sade RM, Hohn A: Cliiical and hemodynamic results of extracardiac conduit reconstruction of the pulmonary artery. Clin Cardiol 3:42, 1980. 5. Mair DD, Fulton RE, Danielson GK: Thrombotic occlusion of Hancock conduit due to severe dehydration after Fontan operation. Mayo Clin Proc 53:397, 1978. 4.
Hypertrophic cardiomyopathy and complete heart block in infancy+ Barry J. Maron, M.D., Thomas M. Connor, M.D., Capt. (MC), USNR, and William C. Roberts, M.D. Bethesda, Md. Hypertrophic cardiomyopathy (HCM) is a congenital malformation that usually does not present clinically until adulthood.l, z However, HCM may be identified during the first year of life3m3 when it often manifests marked congestive heart failure (CHF).s While complete heart block (CHB) due to congenital conducting tissue disorde+ 7 may occur during the first few weeks of life, it is not usually associated with clinical deterioration during infancy.x. * This communication describes the unusual association of HCM and congenital CHB in an infant. A 2-month-old girl had weighed 2.8 kg at term birth by a noninsulin-dependent diabetic mother; CHB with heart rate 40 beats/minute (bpm) (Fig. 1) was known since the first day of life. Severe CHF developed by 3 days of age and an epicardial pacemaker was implanted at a ventricular rate of 100 bpm; digitalis and furosemide were begun. Cardiac catheterization showed left ventricular (LV) pressure 95/12 mm Hg without systolic gradient or mitral regurgitation; LV contractile pattern was normal with an ejection fraction of 0.70. CHF worsened, respiratory failure ensued, and her last month of life was further complicated by disseminated intravascular coagulation. Necropsy heart weight was 62 gm (normal 23 gm).l” The ventricular septum was disproportionately thickened relative to LV free walp’, ” (Fig. 2); septal thickness was 8 mm and LV free wall thickness was 3 mm (ratio 2.7). The coronary arteries were normal and there was no myocardial fibrosis or associated malformations. Marked disorganization of cardiac muscle cells was present in the ventricular septum (Fig. 3); longitudinal cut muscle fibers oriented at oblique and perpendicular angles to each other occupied 17% of. myocardium analyzed.13 LV free wall
From the Cardiology and Pathplogy Branches, Nationa Heart, Lung and Blood Institute, National Institutes of Health, and the Pediatric Cardiology Sectmn, Department of Pediatrics, National Naval Medical Center and United States Uniformed Health Services Medical School. Received
for publication
Feb. 20, 1981; accepted
Feb. 27, 1981.
Reprint requests: Barry J. Maron, M.D., Cardiology Bldg. 10, Room 7B-15, Bethesda, MD 20205.
Branch,
*The opinions and assertions expressed are those of the authors reflect the official position of the Navy.
0002-8703/81/060857
+ 04$00.40/0~1981
NHLBI, and do not
The C.V.Mosby
Co.
Fig. I. Rhythm strip showing rate of 120 bpm.
complete
atrioventricular
Fig. 2. Heart of the patient with hypertrophic cardiomyopathy and congenital complete heart blocky The ventricular septum (VS,Jis substantially thicker than the left ventricular free wall (LVJ Note the large left ventriccavity. ular papillary muscles. RV = right ventricular cardiac fibers were normally arranged. The AV node area was serially sectioned at 6 pm thickness and every tenth section was stained with hemotoxylin and eosin or elastic van Gieson. AV nodal tissue was normal; however, continuity of the conduction system was interrupted in the penetrating portion of the AV bundle (bundle of His) as it coursed through the central fibrous body (Fig. 4). Distal to this area of discontinuity the conducting tissue was normal and the AV bundle divided normally into left and right bundle branches. Because of necropsy findings, M-mode and two-dimensional echocardiograms were obtained in the infant’s parents. The mother’s echogram was normal but the father had asymmetric hypertrophy of the ventricular septum; septal thickness was 18 mm and LV free wall thickness was 10 mm (ratio 1.8). Congenital CHB most commonly occurs in structurally normal bearts, but 30% to 50% of such CHB is associated with a variety of congenital cardiovascular mafforma-
block with ventricular
rate of 40 bpm and atria1
tions.s-a Our patient demonstrates the previously undescribed association of congenital CHB and HCM CHB has been reported previously in a few patients with HCM1d-*l; however, since the age at which CHB was identified in those patients ranged from 10 to 71 years, it is unlikely that CHB was congenital. Our patient appears to have the typical genetically transmitted form of HCMZz: (1) disproportionate ventricular septal thickening,*‘, *’ (2) marked disorganization of cardiac muscle cells in the ventricular septum,lZ and (3) echocardiographic identification of asymmetric hypertrophy of the ventricular septum in the patient’s father.ZZ CHB in our patient appears to have emanated from a developmental defect in the conduction system-discontinuity in the AV bundle at the level of the central fibrous body. This anatomic abnormality of conducting tissue has not been reported in HCM,z5 but has been described m infants with congenital CHB and other congenital heart malformations.6 Whether the conduction system defect in our patient is a morphologic component of HCM or an etiologically independent abnormahty can not be determined with certainty at this time. The patient’s clinical course suggests that CHB and slow ventricular rate may not have contributed substantially to her clinical deterioration and eventual death. The patient’s CHF progressed markedly even after ventricular that our patient’s pacemaker insertion. The probability clinical deterioration was due primarily to the underlying cardiomyopathic process is supported by our previous presents with UZlF in experience that HClVl commonly the first year of life and leads to death in a substantial percentage of such patients5 This patient emphasizes the broad morphologic spectrum of HCM and suggests that HCM should be considered m an infant with CHB and CHF. REFERENCES
I.
2.
3.
4.
Frank S, Braunwald E: Idiopathic hypertrophic subaortic stenosis. Clinical analysis of 126 patients with emphasis on the natural history. Circulation 37:759, 1968. Shah PM, Adelman AG, Wigle ED, Gobel FL? Burchell HB, Hardarson T, Curie1 R, De la Calzada C, Qakley CM, Goodwin JF: The natural (and unnatural) course of hypertrophic obstructive cardiomyopathy. A multicenter study. Circ Res 34 and 35(suppl II):II-179, 1973. Barr PA, Celermajer JM, Bowdler JD, Cartmill TB: Idiopathic hypertrophic obstructive cardiomyopathy causing severe right ventricular outflow tract obstruction in infancy. Br Heart J 35:1109, 1973. Maron BJ, Edwards JE, Henry WL, Clark CE, Bingle GJ,
Volume 101 Number 6
Brief
Fig. 3. Area of ventricular septum showing disorganized arrangement of longitudinally cut cardi,ac muscle cells are oriented obliquely (Hematoxylin and eosin stain; original magnification x 100).
5.
6. 7.
8. 9.
10. 11.
12.
13.
14.
15
16.
Epstein SE: Asymmetric septal hypertrophy (ASH) in infancy. Circulation 50:809, 1974. Maron BJ, Tajik AJ, Ruttenberg HD, Graham TP, Atwood GF, Victorica BE, Lie JT, Roberts WC: Hypertrophic cardiomyopathy in infants: Clinical features and natural history. Circulation (In press) Lev M: Pathogenesis of congenital atrioventricular block. Progr Cardiovasc Dis 15:145, 1973. Jades TN, McKone RC, Hudspeth AS: De Subitaneis Mortibus X. Familial congenital heart block. Circulation 5i :379, 1975. Nakamura FF, Nadas AS: Complete heart block in infants and children. N Engl J Med 270:1261. 1964. Michai%son M, Eigle MA: Congeiital complete heart block: An international study of the natural history. Cardiovast Clin 4:86, 1972. Gould SE: Patlhology of the heart and blood vessels. 3rd ed. Springfield, Ill., 1968, Charles C Thomas, p 1139. Menges H, Brandenburg RO, Brown AL: The clinical, hemodynamic and pathologic diagnosis of muscular subvalvular aortic stenosis. Circulation 24:1126, 1961. Henry WL, Clark CE, Epstein SE: Asymmetric septal hypertrophy (ASH): Echocardiographic identification of the pathognomonic anatomic abnormality of IHSS. Circulation 47:225, 1973. Maron BJ, Satso N, Roberts WC, Edwards JE, Chandra RS: Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum: Comparison of fetuses and infants with and without congenital Reart disease and patients with hypertrophic cardiomyopathy. Circulation 60:685, 1979. Luisada AA: Subaortic muscular stenosis and complete heart block in an adolescent. Chicago Med School Q 25:169, 1965. Matlof HJ, Zener JC, Harrison DC: Idiopathic hypertrophic subaortic stenosis and heart block. Cycle-to-cycle variation as a functi& of alterations in preload and afterload. Am J Cardiol32:719? 1973. Johnson AD, Daily PO: Hypertrophic subaortic stenosis complicated by high degree heart block: Successful treatment with an atria1 synchronous ventricular pacemaker. Chest 67:491, llS75.
Conmunication~
959
of the cardiac muscle cells. Bundles and perpendicularly to each other.
Fig. 4. A section of the atrioventricular bundle area showing interruption in the penetrating portion of the bundle at the level of the central fibrous body. The two unconnected portions of conducting tissue are outlined by broken lines. VS = ventricular septum.
860 17.
18.
19.
20.
Brief
Communications
Spilkin S, Mitha AS, Matisonn RE, Chesler E: Complete heart block in a case of idiopathic hypertrophic subaortic stenosis. Noninvasive correlates with the timing of atria1 systole. Circulation 55:418, 1977. ChmieIewski CA, Riley RS? Mahendran A, Most AS: Complete beart block as a cause of syncope in asymmetric septal hypertrophy. AM HEART J 93:91, 1977. Tajik AJ, Guiliani ER, Frye RLY MeGoon DC, Nunn SL: Muscular subaortic stenosis witR complete heart biock. Am J Cardiol 31:101, 1973. Gavtilescu S, Gavrilescu M, Streian C, Luca C: Hypertrophic obstructive cardiomyopathy associated with complete heart block. Pathologic correlations in a case studied with His bundle electrography~ Acta Cardiol (Brux) 29:241,
1974. 21.
22.
23.
24.
25.
Gilgenkrantz JM, Cherrier F, Petitier H, Dodinot %, Houplan M, Legoux J: Cardiomyopathie obstructive du ventricule gauche avec bloc auriculo-ventriculaire complet; ConsideraGons thkrapeutiques. Arch Ma1 Coeur 61:4i9, 1968. Clark CE. Henrv WL. Erxtein SE: Familial mevalence and genetic tiansmi&ion ‘of -idiopathic hypertriphic subaortic stenosis. N Engl J Med 289:709, 1973. Maron BJ, Henry WL, Roberts WC, Epstein SE: Comparison of echocardiographic and necropsy measurements of ventricular wall thicknesses in patients with and without disproportionate septal thickening. Circulation 55~341, 1977. Henry WL, Ware J, Gardin Jl@, Hepner SI, &Kay J, Weiner M: Echocardiograpbic measurements in normal subjects. Growth-related changes that occur between infancy and early adulthood. Circulation 5?:278, 1978. James TN, Marshall TK: De Subitaneis Mortibus XII. Asymmetric hypertrophy of the heart. Circulation 51:1149, 19%.
Anomalous drainage to through the innominate syndrome
kft pulmonary venous the inferior wena cava and pericardiophrenic vein to the veinLeft-sided Scimitar
M. K. Mardini, M.D., N. A. Sakati, M~D.j and W. L. Nyhan, MOD.? PhD. Riyadh, Saudi Arabia7 and La Jolla, Calif. The term “Scimitar syndrome” was first used by Neil1 et a1.l to describe a disorder with a characteristic roentgenographic appearance in which there was anomalous pulmonary venous (PV) return from the right lung to the inferior vena cava (IVC) in a patient with a hypoplastic right lung and systemic arterial supply to the right lower lobe. The syndrome has since been considered a right-sided malformation. D’Cruz and Arcillaz reported an abnormal PV From the Department of Pediatrics at the King Faisa.1 Specialist Hospital and Resea~h Centus, Riyadh; and the Department of Pediatrics at the University of California San Diego. Reprink requests: Dr. M. K. Mardini, Dept. of Pediatrics, King F&al Speck&it Hospital and Research Centre, P.O. Box 3354, Riyadh, Saudi Arabia.
0002-8703/81/060860
+ 03$00.30/O
0 1981 The
c. V. Mosby
Co.
IV APV PCPV MS
lnnominafe Vein Anomalous Pulmonary Vein Pericardiophrenic Vein Moslem Sword
Fig. IA. Diagrammatic representation in the anterioposterior (A-P) view of anomalous venous connection from the left lung to the inferior vena cava (IVC), and via the pericardiophrenic vein to the innominate vein. The Moslem sword placed alongside provides a reminder of the similarity of its shape to that of the roentgenographic appearance of the anomalous pulmonary vein. SVC = SW perior vena cava; RA = right atrium; PA = pulmonary artery; A = aorta.
connection from a hypoplastic left lung to the IVC. It is the purpose of this report to present the second instance of this condition, and the first in which the anomalous left PV was found to drain into the IVC and into the innominate vein via the pericardiophrenic vein. A 2%-month-old Syrian male infant was referred for bronchoscopy because of a cough and pulmonary density. On examination he appeared well. X-rays showed hypoplasia or partial collapse of the left lung with hyperexpansion of the right, and herniation across the midline. A left-sided scimitar-shaped shadow was visualized in the left lower lung field crossing posterior to the heart and approaching the diaphragm at a 45-degree angle. Tomography was confirmatory. Lung scan with technicium-99 showed decreased perfusion of the left lung, more prominent in the left lower lung field. On ECG there was an RSR prime pattern on lead V, and transitional zone deviation to the left. Cardiac catheterization revealed a minimal step-up of oxygen saturation from the lower IVC to the right atrium and a slight increase in pulmonary artery (PA) pressure. PA angiogram showed a small hypoplastic left PA with abnormal distribution of blood to the left lung. The PV of the left lung joined together to