- Email: [email protected]
Holmes heart in the adult: transesophageal echocardiographic findings and long-term natural survival
International
Journal
of
cardiology ELSEVIER
International Journal of Cardiology 56 (1996) 301-305
Holmes heart in the adult: transesophageal echocardiographic findings and long-term natural survival Antonio Vitarelli” , Fulvio Gabbarini Cardiac Department,
‘LA Sapienza’ Universiq,
Rome, Italy
Received 28 May 1996; revised 23 July 1996: accepted 23 July 1996
Abstract We describe a 59-year-old patient with Holmes heart (single ventricle with normally related great arteries). To our knowledge, this is the oldest patient in the literature with this rare congenital heart diseaseand the oldest one with single ventricle and unrestricted pulmonary blood flow who has developed Eisenmenger’s physiology. A transesophageal echocardiographic examination showed a single large ventricle that was a morphologic left ventricle with a normally appearing aorta originating from this chamber and a normally related pulmonary artery arising from a hypoplastic right infundibulum located anteriorly-superiorly and to the left of the left ventricle. The cardiac catheterization confirmed the echocardiographic findings and revealed a pulmonary artery pressureof 160/60 mmHg. There were no systolic gradients across the pulmonary and aortic outflow tracts. The estimated pulmonary vascular resistance was 2720 dynes-set-cm- ‘. Oximetry showed oxygen saturation in the left ventricle of 84% and femoral artery saturation of 77.7%. Our report further demonstratesthat pulmonary stenosis is not the ultimate determinant of prolonged natural survival in patients with single ventricle. Since this patient is too old for cardiopulmonary transplantation, we plan to follow him conservatively with medical therapy. Keywords:
Congenital heart disease;Single ventricle; Transesophagealechocardiography
1. Introduction The development of new surgical approaches to single ventricle (car triloculare biatriatum) has made it important to know the long-term outcome in unoperated patients. Although this congenital heart disease may result in death in infancy or childhood, a selected group of patients reach adulthood [1,2] and *Corresponding author, via Lima 35 00198 Rome Italy. Tel: 06/85301427; fax: 06/8841926; e mail: [email protected]
in fact single ventricle is one of the commoner complex malformations seen in adults. Most often it is associated with transposed great arteries. To our knowledge, the oldest patients described with single ventricle and transposition of the great arteries were 62 years of age with associated pulmonary stenosis [3] and 31 years of age with no appreciable pulmonic stenosis [4]. A more rare variety of single ventricle is the anomaly usually called ‘Holmes heart’, [S] in which the great arteries are normally related. We recently studied a man with Holmes heart and
0167.5273/96/$15.00 0 1996 Elsevier Science Ireland Ltd. All rights reserved PII SO167-5273(96)02783-O
302
A. Vitarelli, F. Gabbarini I lntemationul
unrestricted pulmonary blood flow who naturally survived into the sixth decade of life.
2. Case report
A 59-year-old man with congenital heart disease was referred for cardiovascular assessment.He was born of an uncomplicated pregnancy and had a precordial murmur first noted at birth. There was no history of heart disease in the patient’s family. He was free of symptoms and his growth and development were considered normal. The first cardiac catheterization was performed at the age of 18 years, when a diagnosis of complex congenital heart disease was made (records not available). No surgery was done. The patient enjoyed playing sports, had worked for years as a taylor and had two children. Except for minimal symptoms,he was relatively well until 6 months being seen by us, when he began to experience more severe exertional dyspnea. On examination the patient was a thin-framed, not dehydrated, cyanosedman. Blood pressurewas 155/ 80 mmHg, pulse was 80 beats/mm, and temperature was 37.1”C. The lungs were clear except for distant breath sounds. Cardiovascular examination revealed a very narrow splitting of the second sound, with a loud S, in the pulmonic area, and a Grade 3 holosystolic precordial murmur with radiation to the axilla. The edge of the liver was felt 2 cm, and the edge of the spleen 1 cm below the costal margin. No peripheral edema was found. Laboratory results showed a hematocrit of 63%, a hemoglobin of 23.6 g/dl, and a room air arterial blood gas with a pH of 7.36, Pco, of 33 mmHg, PO, of 44 mmHg, and
Fig. 1. Multiplane transesophagealcolor flow imaging. Doubleinlet left ventricle is shown with both atria connectedto the same ventricle via two atrio-ventricular valves (higher panel). Scanning low esophageal views, the relationship and connections of great arteries are defined. The aorta with coronary arteries appears originating from the large posterior ventricular chamber (middle panel). Anteriorly and to the left, a normally related pulmonary artery comes off from a rudimentary anterior outlet chamber (lower panel). Ao, aorta; DILV, double-inlet left ventricle; LA, left atrium; LAV, left atrio-ventricular valve; MPA, main pulmonary artery; DC, outlet chamber; RA, right atrium; RAV, right atrioventricular valve.
Journal qf C’mdiolo,~~ 56 (I 996) .Wl -.I05
A. Vitarelli, F. Gabbarini I International
Journal
oxygen saturation of 78%. An electrocardiogram showed sinus rhythm with biventricular hypertrophy. A chest X-ray found cardiomegaly with increased pulmonary vascularity. Pulmonary function testing revealed reduced total lung capacity (69% of predicted) and forced vital capacity (65% of predicted). Due to the paucity of transthoracic echo windows, a transesophageal echocardiographic examination (Fig. 1) was performed that showed a single large ventricle that was a morphologic left ventricle with a normally appearing aorta originating from this ventricle. There was anterograde flow from the morphologic left ventricle through a nonrestrictive bulboventicular foramen into a hypoplastic right infundibulum which lay anterior-superior and to the left of the left ventricle, with a normally related (anterior) pulmonary artery originating from this rudimentary chamber. The pulmonary trunk was dilated and there was a high velocity jet from pulmonary insufficiency. No systolic gradient across the pulmonary outflow tract was demonstrated. Doppler estimated systolic pulmonary artery pressure was 150 mmHg. There were two intact atria with two normally located atrioventricular valves connecting with the common ventricle and there was mitral aortic continuity. Mild left A-V valve regurgitation was shown. Ventricular fractional area change was 0.47 and ventricular ejection fraction (by the method of discs) was 0.58. A cardiac catheterization was done to confirm the echocardiographic findings. A pulmonary artery pressure of 160/60 mmHg was shown (Table 1). There were no systolic gradients across the pulmonary and aortic outflow tracts. The estimated pulmon-
of Cardiology 56 (1996) 301-305
303
ary vascular resistance was 2720 dynes-set-cm-‘. Oximetry showed oxygen saturation in the left ventricle of 84% and femoral artery saturation of 77.7%.
3. Discussion Our report describes the oldest patient in the literature with Holmes heart and points out the diagnostic information provided by transesophageal echocardiography in this complex congenital heart disease. Dr Holmes considered his patient’s congenital anomaly as ‘an intermediate form between cases of imperfect double heart and those of single heart’ [5]. Similar hearts described by other authors [6-91 all have both atrial chambers connected to the morphologically left ventricle in the presence of a rudimentary right ventricle. In all cases the aorta was connected to the left ventricle and the pulmonary trunk to the right ventricle (ventriculoarterial concordance). Some hearts with this segmental combination have also been described as ‘displaced tricuspid orifice’ [7], ‘single ventricle type Al’ [8] or ‘doubleinlet left ventricle with rudimentary right ventricle and ventriculoarterial concordance’ [9]. The anomaly is of significance with regard to its differential diagnosis and the disposition of the conducting tissues much closer to the posterior surface of the heart. Holmes heart was a very rare event in a large series of autopsied hearts (Saroja Bharati, personal communication). In adolescents and adults with congenital heart
Table 1 Cardiac catheterization data Parameter
Pressures (mmHg)
Mean
WC IVC
RA MPA PC (wedge) WC Ao FA
160160 160/O/ 14 160160 160/60
8 95 8
0, saturation (%)
0, content (ml/l)
51 66.7 61 72.1
19.3 22.6 20.1 25
84.1 78.2
28.5 26.5 26.1
71.1
HR (beats/tin)
80
Ao, ascending aorta; FA, femoral artery; HR, heart rate; IVC, inferior vena cava; MPA, main pulmonary artery; PC, pulmonary capillaries; RA, right atrium; WC, superior vena cava; UVC, univentricular chamber.
304
A. Vitarelli, F. Gabbarini I International
disease the diagnostic information provided by transthoracic echocardiography is often incomplete because of the larger body, associated chest deformities, and paucity of echo windows [lo]. Transesophageal echocardiography is an unrivaled method for examining the atrioventicular valves with their chordal attachments, semilunar valves, atria1 septum, and appendages. The addition of the longitudinal plane has made an important contribution to the evaluation of the right ventricular outflow tract, pulmonary valve and main pulmonary artery. A nice visualization of pulmonary valve morphology and motion and evaluation of absence of obstruction at any level (valvar, subvalvar) were achieved in our patient (Fig. 1). Compared to biplane technology, the multiplane f%element probe allowed better resolution as well as more reliable pulsed and continuous Doppler interrogation of the pulmonary trunk and right ventricular outflow tract in multiple examination planes. Thus the use of multiplane TEE can minimize the problem of alignment with jets for velocity measurements and provide important pathophysiological information in complex congenital heart disease. The cardiologist performing TEE in congenital heart disease should be familiar with the various congenital defects to be able to interpret these findings in real time. Congenital diseases of the heart and circulation are not fixed anatomic defects but dynamic anomalies that originate in fetal life and change considerably during postnatal development and throughout childhood, adolescence, and adulthood. Enhanced survival in single ventricle depends primarily on factors that limit the volume overload of the pulmonary circuit, such as the presence of pulmonary stenosis or an increase in pulmonary vascular resistance [ 1,2]. When pulmonary resistance is low and pulmonary stenosis is absent, life expectancy is short. Excessive pulmonary arterial blood flow results in left ventricular failure unless the pulmonary vascular resistance rises appropriately. On the other hand, with excessive pulmonic stenosis, hypoxia and cyanosis results. Additional variables include the size of interventricular communication, the great arteries anatomy, the functional adequacy of the atrioventricular connections, and the morphologic type of single ventricle [9- 111. A favorable combination of these parameters allows a balance between a decrease of pulmonary
Joumal cf Cardiology -56 (1996) 701-30.5
blood flow to relieve volume overload and sufficient pulmonary blood flow to oxygenate systemic circulation. Our report further demonstrates that pulmonary stenosis is not the ultimate determinant of prolonged natural survival in patients with single ventricle. Physiologic palliation may be obtained in spite of a severe degree of pulmonary hypertension. Intraventricular streaming must occur with preferential movement of oxygenated blood from the left atrium into the systemic circulation. Previous reports showed preferential streaming of oxygenated blood from the left atrium into the aorta in a patient with single ventricle, pulmonic stenosis and L-transposition of the great vessels [3] or ‘contralateral’ streaming effect in a patient with single ventricle, pulmonary hypertension, displaced right atrioventricular connection and D-transposition of the great vessels [4]. Unlike their experience, our case had single ventricle with normally related great vessels and unrestricted pulmonary blood flow. The normal anatomy of the great arteries allowed for streaming to occur and explains the moderate arterial desaturation at rest despite pulmonary hypertension and high pulmonary vascular resistance. Our patient is the oldest with single ventricle who has developed Eisenmenger’s physiology. An abnormal ventilatory response with mild restrictive lung disease as he presented has also been described in patients with primary pulmonary hypertension or cyanotic congenital heart disease [ 12- 141. Interstitial fibrosis and degenerative changes in myocites are common in adults with congenital heart disease even in the absence of signs of heart failure [15] and can cause a subtle deterioration of ventricular function. Since this patient is too old for cardiopulmonary transplantation, we plan to follow him conservatively with medical therapy.
References [ 11 Perloff JK, Child IS. Congenital heart disease in adults. Philadelphia: WB Saunders, 1991; 44. [2] Liberthson RR. Congenital heart disease. Diagnosis and management in children and adults. Boston: Little, Brown and Company, 1989; 141-148. [3] Goldberg HL, Sniderman K, Devereux RB, Levin A. Prolonged survival (62 years) with single ventricle. Am J Cardiol 1983; 52: 214-215.
A. Vitarelli, F. Gabbarini I International [4] Steinberg EH, Dantzker DR. Single ventricle with severe pulmonary hypertension: natural survival into the third decade of life. Am Heart J 1993; 125: 1451-1453. [5] Holmes AF. Case of malformation of the heart. Tram Med Chir Sot Edinb 1824; 1: 252-259. [6] Lev M, Liberthson RR, Kirkpatrick JR, Eckner F.A.O., Arcilla R.A. Single (primitive) ventricle. Circulation 1969; 39: 577-591. ]7] Bharati S, Lev M. The relationship between single ventricle and small outlet chamber and straddling and displaced tricuspid orifice and valve. Herz 1979; 4: 176-183. [8] Van Praagh R, Ongley PA, Swan HJC. Anatomic types of single or common ventricle in man: morphologic and geometric aspects of sixty necropsied cases. Am J Cardiol 1964; 13: 367-386. [9] Anderson RH, Lenox CC, Zuberbuhler RJ, HO SY, Smith A, Wilkinson JL. Double-inlet left ventricle with rudimentary right ventricle and ventriculoarterial concordance. Am J Cardiol 1983; 52: 573-577. [lo] Hirsch R, Kilner PJ, Connelly MS, Redington AN, St John Sutton MG, Somerville J. Diagnosis in adolescents and adults with congenital heart disease. Prospective assessment of individual and combined roles of magnetic resonance imaging and transesophageal echocardiography. Circulation 1994; 90: 2937-2951.
Journal of Cardiology
56 (1996) 301-305
305
[l l] Shiaraishi H, Silverman NM. Echocardiographic spectrum of double inlet ventricle: evaluation of the interventricular communication. J Am Co11 Cardiol 1990; 15: 1401-1408. [12] Discroll DJ, Staats BA, Heise CT, Rice MJ, Puga FJ,, Danielson GK, Ritter DJ. Functional single ventricle: car diorespiratory response to exercise. J Am Co11Cardiol 1984: 4: 337-342. [ 131 Rich S, Dantzker DR, Ayers SM. Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldbring RM, Groves BM, Koemer SK, Levy PC, Reid LM, Vreim CE, Willimans GW. Primary pulmonary hypertension. Ann Intern Med 1987; 107: 216-223. [14] Sandoval J, Bauerle 0, Gomez A, Palomar A, Martinez Guerra ML, Furuya ME. Primary pulmonary hypertension in children: clinical characterization and survival. J Am Co11 Cardiol 1995; 25: 466-474. [15] Hopkins WE, Waggoner AD, Gussak H. Quantitative ultrasonic tissue characterization of myocardium in cyanotic adults with an unrepaired congenital heart defect. Am J Cardiol 1994; 74: 930-934.
Journal
of
cardiology ELSEVIER
International Journal of Cardiology 56 (1996) 301-305
Holmes heart in the adult: transesophageal echocardiographic findings and long-term natural survival Antonio Vitarelli” , Fulvio Gabbarini Cardiac Department,
‘LA Sapienza’ Universiq,
Rome, Italy
Received 28 May 1996; revised 23 July 1996: accepted 23 July 1996
Abstract We describe a 59-year-old patient with Holmes heart (single ventricle with normally related great arteries). To our knowledge, this is the oldest patient in the literature with this rare congenital heart diseaseand the oldest one with single ventricle and unrestricted pulmonary blood flow who has developed Eisenmenger’s physiology. A transesophageal echocardiographic examination showed a single large ventricle that was a morphologic left ventricle with a normally appearing aorta originating from this chamber and a normally related pulmonary artery arising from a hypoplastic right infundibulum located anteriorly-superiorly and to the left of the left ventricle. The cardiac catheterization confirmed the echocardiographic findings and revealed a pulmonary artery pressureof 160/60 mmHg. There were no systolic gradients across the pulmonary and aortic outflow tracts. The estimated pulmonary vascular resistance was 2720 dynes-set-cm- ‘. Oximetry showed oxygen saturation in the left ventricle of 84% and femoral artery saturation of 77.7%. Our report further demonstratesthat pulmonary stenosis is not the ultimate determinant of prolonged natural survival in patients with single ventricle. Since this patient is too old for cardiopulmonary transplantation, we plan to follow him conservatively with medical therapy. Keywords:
Congenital heart disease;Single ventricle; Transesophagealechocardiography
1. Introduction The development of new surgical approaches to single ventricle (car triloculare biatriatum) has made it important to know the long-term outcome in unoperated patients. Although this congenital heart disease may result in death in infancy or childhood, a selected group of patients reach adulthood [1,2] and *Corresponding author, via Lima 35 00198 Rome Italy. Tel: 06/85301427; fax: 06/8841926; e mail: [email protected]
in fact single ventricle is one of the commoner complex malformations seen in adults. Most often it is associated with transposed great arteries. To our knowledge, the oldest patients described with single ventricle and transposition of the great arteries were 62 years of age with associated pulmonary stenosis [3] and 31 years of age with no appreciable pulmonic stenosis [4]. A more rare variety of single ventricle is the anomaly usually called ‘Holmes heart’, [S] in which the great arteries are normally related. We recently studied a man with Holmes heart and
0167.5273/96/$15.00 0 1996 Elsevier Science Ireland Ltd. All rights reserved PII SO167-5273(96)02783-O
302
A. Vitarelli, F. Gabbarini I lntemationul
unrestricted pulmonary blood flow who naturally survived into the sixth decade of life.
2. Case report
A 59-year-old man with congenital heart disease was referred for cardiovascular assessment.He was born of an uncomplicated pregnancy and had a precordial murmur first noted at birth. There was no history of heart disease in the patient’s family. He was free of symptoms and his growth and development were considered normal. The first cardiac catheterization was performed at the age of 18 years, when a diagnosis of complex congenital heart disease was made (records not available). No surgery was done. The patient enjoyed playing sports, had worked for years as a taylor and had two children. Except for minimal symptoms,he was relatively well until 6 months being seen by us, when he began to experience more severe exertional dyspnea. On examination the patient was a thin-framed, not dehydrated, cyanosedman. Blood pressurewas 155/ 80 mmHg, pulse was 80 beats/mm, and temperature was 37.1”C. The lungs were clear except for distant breath sounds. Cardiovascular examination revealed a very narrow splitting of the second sound, with a loud S, in the pulmonic area, and a Grade 3 holosystolic precordial murmur with radiation to the axilla. The edge of the liver was felt 2 cm, and the edge of the spleen 1 cm below the costal margin. No peripheral edema was found. Laboratory results showed a hematocrit of 63%, a hemoglobin of 23.6 g/dl, and a room air arterial blood gas with a pH of 7.36, Pco, of 33 mmHg, PO, of 44 mmHg, and
Fig. 1. Multiplane transesophagealcolor flow imaging. Doubleinlet left ventricle is shown with both atria connectedto the same ventricle via two atrio-ventricular valves (higher panel). Scanning low esophageal views, the relationship and connections of great arteries are defined. The aorta with coronary arteries appears originating from the large posterior ventricular chamber (middle panel). Anteriorly and to the left, a normally related pulmonary artery comes off from a rudimentary anterior outlet chamber (lower panel). Ao, aorta; DILV, double-inlet left ventricle; LA, left atrium; LAV, left atrio-ventricular valve; MPA, main pulmonary artery; DC, outlet chamber; RA, right atrium; RAV, right atrioventricular valve.
Journal qf C’mdiolo,~~ 56 (I 996) .Wl -.I05
A. Vitarelli, F. Gabbarini I International
Journal
oxygen saturation of 78%. An electrocardiogram showed sinus rhythm with biventricular hypertrophy. A chest X-ray found cardiomegaly with increased pulmonary vascularity. Pulmonary function testing revealed reduced total lung capacity (69% of predicted) and forced vital capacity (65% of predicted). Due to the paucity of transthoracic echo windows, a transesophageal echocardiographic examination (Fig. 1) was performed that showed a single large ventricle that was a morphologic left ventricle with a normally appearing aorta originating from this ventricle. There was anterograde flow from the morphologic left ventricle through a nonrestrictive bulboventicular foramen into a hypoplastic right infundibulum which lay anterior-superior and to the left of the left ventricle, with a normally related (anterior) pulmonary artery originating from this rudimentary chamber. The pulmonary trunk was dilated and there was a high velocity jet from pulmonary insufficiency. No systolic gradient across the pulmonary outflow tract was demonstrated. Doppler estimated systolic pulmonary artery pressure was 150 mmHg. There were two intact atria with two normally located atrioventricular valves connecting with the common ventricle and there was mitral aortic continuity. Mild left A-V valve regurgitation was shown. Ventricular fractional area change was 0.47 and ventricular ejection fraction (by the method of discs) was 0.58. A cardiac catheterization was done to confirm the echocardiographic findings. A pulmonary artery pressure of 160/60 mmHg was shown (Table 1). There were no systolic gradients across the pulmonary and aortic outflow tracts. The estimated pulmon-
of Cardiology 56 (1996) 301-305
303
ary vascular resistance was 2720 dynes-set-cm-‘. Oximetry showed oxygen saturation in the left ventricle of 84% and femoral artery saturation of 77.7%.
3. Discussion Our report describes the oldest patient in the literature with Holmes heart and points out the diagnostic information provided by transesophageal echocardiography in this complex congenital heart disease. Dr Holmes considered his patient’s congenital anomaly as ‘an intermediate form between cases of imperfect double heart and those of single heart’ [5]. Similar hearts described by other authors [6-91 all have both atrial chambers connected to the morphologically left ventricle in the presence of a rudimentary right ventricle. In all cases the aorta was connected to the left ventricle and the pulmonary trunk to the right ventricle (ventriculoarterial concordance). Some hearts with this segmental combination have also been described as ‘displaced tricuspid orifice’ [7], ‘single ventricle type Al’ [8] or ‘doubleinlet left ventricle with rudimentary right ventricle and ventriculoarterial concordance’ [9]. The anomaly is of significance with regard to its differential diagnosis and the disposition of the conducting tissues much closer to the posterior surface of the heart. Holmes heart was a very rare event in a large series of autopsied hearts (Saroja Bharati, personal communication). In adolescents and adults with congenital heart
Table 1 Cardiac catheterization data Parameter
Pressures (mmHg)
Mean
WC IVC
RA MPA PC (wedge) WC Ao FA
160160 160/O/ 14 160160 160/60
8 95 8
0, saturation (%)
0, content (ml/l)
51 66.7 61 72.1
19.3 22.6 20.1 25
84.1 78.2
28.5 26.5 26.1
71.1
HR (beats/tin)
80
Ao, ascending aorta; FA, femoral artery; HR, heart rate; IVC, inferior vena cava; MPA, main pulmonary artery; PC, pulmonary capillaries; RA, right atrium; WC, superior vena cava; UVC, univentricular chamber.
304
A. Vitarelli, F. Gabbarini I International
disease the diagnostic information provided by transthoracic echocardiography is often incomplete because of the larger body, associated chest deformities, and paucity of echo windows [lo]. Transesophageal echocardiography is an unrivaled method for examining the atrioventicular valves with their chordal attachments, semilunar valves, atria1 septum, and appendages. The addition of the longitudinal plane has made an important contribution to the evaluation of the right ventricular outflow tract, pulmonary valve and main pulmonary artery. A nice visualization of pulmonary valve morphology and motion and evaluation of absence of obstruction at any level (valvar, subvalvar) were achieved in our patient (Fig. 1). Compared to biplane technology, the multiplane f%element probe allowed better resolution as well as more reliable pulsed and continuous Doppler interrogation of the pulmonary trunk and right ventricular outflow tract in multiple examination planes. Thus the use of multiplane TEE can minimize the problem of alignment with jets for velocity measurements and provide important pathophysiological information in complex congenital heart disease. The cardiologist performing TEE in congenital heart disease should be familiar with the various congenital defects to be able to interpret these findings in real time. Congenital diseases of the heart and circulation are not fixed anatomic defects but dynamic anomalies that originate in fetal life and change considerably during postnatal development and throughout childhood, adolescence, and adulthood. Enhanced survival in single ventricle depends primarily on factors that limit the volume overload of the pulmonary circuit, such as the presence of pulmonary stenosis or an increase in pulmonary vascular resistance [ 1,2]. When pulmonary resistance is low and pulmonary stenosis is absent, life expectancy is short. Excessive pulmonary arterial blood flow results in left ventricular failure unless the pulmonary vascular resistance rises appropriately. On the other hand, with excessive pulmonic stenosis, hypoxia and cyanosis results. Additional variables include the size of interventricular communication, the great arteries anatomy, the functional adequacy of the atrioventricular connections, and the morphologic type of single ventricle [9- 111. A favorable combination of these parameters allows a balance between a decrease of pulmonary
Joumal cf Cardiology -56 (1996) 701-30.5
blood flow to relieve volume overload and sufficient pulmonary blood flow to oxygenate systemic circulation. Our report further demonstrates that pulmonary stenosis is not the ultimate determinant of prolonged natural survival in patients with single ventricle. Physiologic palliation may be obtained in spite of a severe degree of pulmonary hypertension. Intraventricular streaming must occur with preferential movement of oxygenated blood from the left atrium into the systemic circulation. Previous reports showed preferential streaming of oxygenated blood from the left atrium into the aorta in a patient with single ventricle, pulmonic stenosis and L-transposition of the great vessels [3] or ‘contralateral’ streaming effect in a patient with single ventricle, pulmonary hypertension, displaced right atrioventricular connection and D-transposition of the great vessels [4]. Unlike their experience, our case had single ventricle with normally related great vessels and unrestricted pulmonary blood flow. The normal anatomy of the great arteries allowed for streaming to occur and explains the moderate arterial desaturation at rest despite pulmonary hypertension and high pulmonary vascular resistance. Our patient is the oldest with single ventricle who has developed Eisenmenger’s physiology. An abnormal ventilatory response with mild restrictive lung disease as he presented has also been described in patients with primary pulmonary hypertension or cyanotic congenital heart disease [ 12- 141. Interstitial fibrosis and degenerative changes in myocites are common in adults with congenital heart disease even in the absence of signs of heart failure [15] and can cause a subtle deterioration of ventricular function. Since this patient is too old for cardiopulmonary transplantation, we plan to follow him conservatively with medical therapy.
References [ 11 Perloff JK, Child IS. Congenital heart disease in adults. Philadelphia: WB Saunders, 1991; 44. [2] Liberthson RR. Congenital heart disease. Diagnosis and management in children and adults. Boston: Little, Brown and Company, 1989; 141-148. [3] Goldberg HL, Sniderman K, Devereux RB, Levin A. Prolonged survival (62 years) with single ventricle. Am J Cardiol 1983; 52: 214-215.
A. Vitarelli, F. Gabbarini I International [4] Steinberg EH, Dantzker DR. Single ventricle with severe pulmonary hypertension: natural survival into the third decade of life. Am Heart J 1993; 125: 1451-1453. [5] Holmes AF. Case of malformation of the heart. Tram Med Chir Sot Edinb 1824; 1: 252-259. [6] Lev M, Liberthson RR, Kirkpatrick JR, Eckner F.A.O., Arcilla R.A. Single (primitive) ventricle. Circulation 1969; 39: 577-591. ]7] Bharati S, Lev M. The relationship between single ventricle and small outlet chamber and straddling and displaced tricuspid orifice and valve. Herz 1979; 4: 176-183. [8] Van Praagh R, Ongley PA, Swan HJC. Anatomic types of single or common ventricle in man: morphologic and geometric aspects of sixty necropsied cases. Am J Cardiol 1964; 13: 367-386. [9] Anderson RH, Lenox CC, Zuberbuhler RJ, HO SY, Smith A, Wilkinson JL. Double-inlet left ventricle with rudimentary right ventricle and ventriculoarterial concordance. Am J Cardiol 1983; 52: 573-577. [lo] Hirsch R, Kilner PJ, Connelly MS, Redington AN, St John Sutton MG, Somerville J. Diagnosis in adolescents and adults with congenital heart disease. Prospective assessment of individual and combined roles of magnetic resonance imaging and transesophageal echocardiography. Circulation 1994; 90: 2937-2951.
Journal of Cardiology
56 (1996) 301-305
305
[l l] Shiaraishi H, Silverman NM. Echocardiographic spectrum of double inlet ventricle: evaluation of the interventricular communication. J Am Co11 Cardiol 1990; 15: 1401-1408. [12] Discroll DJ, Staats BA, Heise CT, Rice MJ, Puga FJ,, Danielson GK, Ritter DJ. Functional single ventricle: car diorespiratory response to exercise. J Am Co11Cardiol 1984: 4: 337-342. [ 131 Rich S, Dantzker DR, Ayers SM. Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldbring RM, Groves BM, Koemer SK, Levy PC, Reid LM, Vreim CE, Willimans GW. Primary pulmonary hypertension. Ann Intern Med 1987; 107: 216-223. [14] Sandoval J, Bauerle 0, Gomez A, Palomar A, Martinez Guerra ML, Furuya ME. Primary pulmonary hypertension in children: clinical characterization and survival. J Am Co11 Cardiol 1995; 25: 466-474. [15] Hopkins WE, Waggoner AD, Gussak H. Quantitative ultrasonic tissue characterization of myocardium in cyanotic adults with an unrepaired congenital heart defect. Am J Cardiol 1994; 74: 930-934.