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Prolonged survival despite severe cyanosis in an adult with right ventricular hypoplasia and atrial septal defect
Volume Number
120 3
lungs, with a normal ventilation pattern. A venogram demonstrated an extensive thrombus of the left leg veins extending into the popliteal vein with a patent left iliac vein. A follow-up echocardiogram 1 day later revealed persistently poor biventricular function and no right-sided masses. The patient’s cardiac rhythm ultimately returned to sinus and he was discharged on a regimen of warfarin sodium. One year after admission to the hospital, he was seen for routine follow-up and was found to be back to his baseline activity status. Our case illustrates the sudden, complete, and unpredictable embolization of free-floating thrombus within the right atrium. Lower extremity venography in our case demonstrated that the RATE was probably a single fragment of an even larger deep venous thrombus within the leg veins. The multiple defects seen on the lung perfusion scan may indicate that the visualized embolic event was only one of a series of subacute pulmonary emboli. It is surprising how well-tolerated the pulmonary embolus was, given the patient’s advanced left-sided heart disease. One can only speculate that chronic left ventricular disease may have prepared the right ventricle for variable degrees of increased pulmonary vascular resistance. Presumably, a deep venous thromboembolus will pass rapidly to the lungs if its size is small relative to the tricuspid apparatus. A previous case report by Arvan and DuBois5 documented a shower of multiple small emboli that appeared and persisted in the right atrium for several minutes before passing to the lungs. The thrombus in our patient’s right atrium was large and was present at the onset of imaging. Its chronicity is uncertain, but once the thrombus was across the tricuspid valve, it passed rapidly to the pulmonary circulation without fragmentation. The percentage of thromboemboli large enough to be held up within the right atrium is unknown. When echocardiography is performed prospectively in patients diagnosed with pulmonary embolism, RATE are infrequently found (incidence less than 1 %).6 However, prior to embolization, RATE are typically unsuspected, are clinically occult, and are often found on incidental echocardiography. Although the true frequency of RATE can never be known, the clinical implications of their detection in the ultrasound laboratory are quite clear. They are the echocardiographic harbinger of a large, often fatal, pulmonary embolus.‘? 4 This case report documents for the first time the actual passage of a single, large, unattached thrombus within the right atrium to the pulmonary circulation. The clinical course of this patient is notable for the unpredictability of embolization in this setting, the absence of symptoms noted following the visualized embolization, and the seeming e0icacy of anticoagulation after embolization without hemodynamic instability. REFERENCES
1. Kinney EL, Wright RJ. Efficacy of treatment of patients with echocardiographically detected right-sided heart thrombi: a meta-analysis. AM HEART J 1989;118:569-73. 2. Panidis IP, Kotler MN, Mintz GS, Ross J. Clinical and
Brief Communications
701
echocardiographic features of right atria1 masses. AM HEART J 1984;107:745-58. Saner HS, Asinger RW, Daniel JA, ElspergerKJ. Twodimensional echocardiographic detection of right-sided cardiac intracavitary thromboembolus with pulmonary embolism. J Am Co11 Cardiol 1984;4:1294-301. Farfel Z, Shechter M, Vered Z, Rath S, Goor D, Gafni J. Review of echocardiographically diagnosed right heart entrapment of pulmonary emboli-in-transit with emphasis on management. AM HEART J 1987;113:171-8. Arvan S, DuBois B. M-mode and two-dimensional echocardiography of pulmonary emboli. J Clin Ultrasound 1982;10:177-9. Kasper W, Meinertz T, Henkel B, Eissner D, Hahn K, Hofmann T, Zeiher A, Just H. Echocardiographic findings in patients with proved pulmonary embolism. AM HEART J 1986;112:1284-90.
Prolonged survival despite severe cyanosis in an adult with right ventricular hypoplasia and atrial septal defect Dean G. Karalis, MD, K. Chandrasekaran,MD, Mark F. Victor, MD, and Gary S. Mintz, MD. Philadelphia,
Pa.
Isolated right ventricular hypoplasia is an uncommoncardiac malformation. Both the right ventricle and tricuspid valve are underdeveloped. An interatrial communication, most often an atria1 septal defect, is universally present.’ Right-to-left shunting at the atria1 level develops, with normal pulmonary artery pressures.The clinical picture is dominated by right heart failure and cyanosis.The prognosisis generally poor and survival into adult life is rare.2 We report a caseof a 57-year-old man with severecyanosis due to a hypoplastic right ventricle with right-to-left shunting acrossan atria1 septal defect. To our knowledge, this is the oldest describedpatient to have such an anomaly. A 57-year-old man had clubbing and cyanosis since childhood. He had a long history of hypertension and polycythemia and had been receiving long-term hemodialysis therapy for 7 years. Several days prior to this admission, he developedprogressivedyspnea and wasevaluated at another hospital. He wasfound to be cyanotic and in severe congestiveheart failure. The arterial partial pressure of oxygen (POT) was 43 mm Hg on room air and did not appreciably increasewith 100% oxygen. Vigorous hemodialysis resulted in an improvement in pulmonary venous congestion;however, he remained cyanotic. A pulmonary perfusion scanshowedno abnormalities.Pulmonary artery From the Likoff Cardiovascular Institute, Department of Medicine, Hahnemann University Hospital. Reprint requests: K. Chandrasekaran, MD, Hahnemann University Hospital, Cardiac Ultrasound Laboratory, Broad and Vine Streets, Mail Stop 313, Philadelphia, PA 19102.
414122044
September
702
Brief Communications
American
Heart
1990 Journal
Fig. 1. Transesophagealechocardiographicfour-chamber view demonstrating an atria1septal defect (top arrow) and smallright ventricle (RV) and tricuspid valve (bottom arrow). Flow acrossthe atria1 septal defect was right to left. LA, Left atrium; LV, left ventricle; RA, right atrium.
pressurewas32112mm Hg and pulmonary capillary wedge pressurewas 14 mm Hg. He wastransferred to our hospital for further evaluation of refractory hypoxemia. There was central and peripheral cyanosis and finger clubbing. The heart rate was regular at 90 beats/min. The blood pressurewas 120/70 mm Hg. The lungs were clear to auscultation. The secondheart soundwassingleand there was a 2/6 holosystolic murmur at the left sternal border radiating to the apex. There wasno peripheral edema.Arterial blood gasdetermination on 100% oxygen revealed a pH of 7.52,a POZof 46, and a PCO~of 26. The hemoglobinwas15.1 mg/dl. The electrocardiogram demonstrated normal sinus rhythm and first-degree atrioventricular block. The QRS axis was a negative 60 degreesand there were new anterolateral ST and T wave abnormalities suggestive of ischemia. Chest roentgenogramrevealed a mildly enlarged cardiac silhouette and mild pulmonary edema. A transthoracic echocardiogram was performed. The study wastechnically inadequate; however, it revealed left ventricular hypertrophy and severeanterior wall hypokinesis.The right ventricle and tricuspid valve were not well visualized. A transesophagealechocardiogram was performed (Fig. 1). The right ventricle was small in size and the endocardial surface was smooth without trabeculations. The tricuspid valve anulus was small, nonstenotic, and measured 1.5 cm in diameter. These findings were consistent with right ventricular hypoplasia. The pulmonary trunk wasnormal. There wasa large secundumatria1 septal defect. Contrast transesophagealechocardiography demonstrated right-to-left shunting acrossthe atria1 septal defect. The patient underwent cardiac catheterization.
A flow-directed balloon catheter passedeasily through the atria1 septal defect and into the left atrium. The hemodynamic data are displayed in Table I. The shunt acrossthe atria1 septal defect was pure right-to-left. A right ventriculogram demonstrated a small, smooth hypokinetic right ventricle without trabeculations. The left ventriculogram demonstrated anteroapical hypokinesis and mild mitral regurgitation. The left ventricular ejection fraction was 35%. There wasa 70% stenosisover a long portion of the proximal left anterior descendingartery. The left circumflex and dominant right coronary arteries werenormal. The patient’s clinical condition improved; however, his cyanosispersisted. It wasfelt that his initial respiratory decompensationwasdue to pulmonary venous congestionfrom a recent anterior wall myocardial infarction. Since there was no evidencefor residual ischemia,his coronary diseasewas treated medically. In addition, since his cyanosiswaswell tolerated and sincehe had remained asymptomatic for his entire adult life, it waselected not to proceed with operative correction of his congenital heart disease.One year after this admissionhe remains asymptomatic. In adults, cyanosisresulting from shunt reversal at the atrial level rarely occurs with normal pulmonary artery pressures.However, an atria1 septal defect that is part of a complex congenital malformation may result in cyanosis without pulmonary hypertension because of obligatory right-to-left shunting acrossthe atria1 septum. In patients with hypoplasia of the right ventricle, survival is generally poor due to inadequate blood flow to the lungs, becauseof right heart failure, and from the consequencesof marked polycythemia. Survival into adulthood dependson the size
Volume Number
Table
120 3
Brief Communications 703
I. Cardiac catheterization Pressure (mm &?I (mean)
Site Superior vena cava Inferior vena cava Right atrium Right ventricle Pulmonary artery Right pulmonary artery Left pulmonary artery Left atrium Left ventricle Right lower pulmonary vein Left upper pulmonary vein Aorta
(12) 30112 30/14 (18)
02) 110/12 110/70
Oxygen saturation* (%) 64 52 60 59 56 55 62 80 19 96 96 80
*Obtained while patient was breathing 100% oxygen.
of the interatrial communication, the degreeof right ventricular hypoplasia, and the maintenance of adequate blood flow to the lungs.The age of onset of symptoms varies; however, prolonged survival into adult life is rare. Sackner et a1.3describeda patient who survived to the age of 30, dying from refractory right heart failure. The clinical course of our patient is unique due to his prolonged survival into adult life. Furthermore, despite marked cyanosishe has remained relatively asymptomatic. We assumethat this wasdue to multiple physiologic responsesthat preserved tissue oxygenation despite severe cyanosis,such assecondarypolycythemia and increasesin cellular 2,3-DPG (2,3-diphosphoglycerate). However, the greatly increasedhematocrit can lead to undesirableconsequences.In this patient depressederythropoiesis from chronic renal failure led to attenuation of the polycythemic response.In addition, the hypoplastic right ventricle protected the pulmonary vascularbed from increasedflow that occursin a simple atria1 septal defect, but fortuitously the degree of hypoplasia allowed sufficient flow, permitting adequate oxygenation and the prevention of right-sided heart failure. Moreover, it is possiblethat long-standing hypertension in our patient may have played a role in decreasingthe right-to-left shunt, thereby augmentingblood flow through the pulmonary artery. These mechanisms may explain the prolonged survival in our patient. The presenceof cyanosisin an adult with an atrial septal defect usually heralds the development of suprasystemic
pulmonary
hypertension.
Only rarely
has cyanosis
with an atrial septal defect been reported in the presence of normal pulmonary artery pressures.In the present case, cyanosisoccurred despite normal pulmonary artery pressures becauseof restriction of venous inflow by the hypoplastic right ventricle, causingan obligatory right-to-left shunt at the atria1 level. The tricuspid valve, though small, wasnot a significant abnormality; its size simply reflected hypoplasia of the right ventricle. Other anatomic anomalies may favor right-to-left shunting with normal pulmonary artery pressures.Gallaher et a1.4describedthree patients
with
abnormally
large
eustachian
valves
that
extended from the origin of the inferior vena cava to the
margin of the atria1 septal defect, selectively channeling blood into the left atrium. Similarly, congenital anomalous systemic venous connections that terminate in the atria1 wall at the site of the atria1 septal defect result in the return of venous blood into the left atrium.5 After pneumonectomy, cyanosisfrom shunt reversal acrossan atria1 septal defect may occur despite normal pulmonary artery pressures,possibly due to mechanical alterations in right atria1 and ventricular configurations.6 In conclusion,severalpoints in this caserequire specific attention. First, although this malformation is often corrected surgically, prolonged asymptomatic survival despite severe cyanosis may permit a conservative treatment strategy in some patients. Second, not all right-to-left shunting acrossan atria1 septal defect is associated-with severepulmonary hypertension. Although this congenital malformation is rare in an adult population, it and other anatomic anomaliesshould be consideredin the differential diagnosisof a cyanotic adult with an atria1 septal defect and normal pulmonary artery pressures.Finally, in evaluating a patient with congenital heart diseasein whom the transthoracic echocardiogramis not adequate, transesophagealechocardiography should be performed. This is especially important in a sick adult with a potentially complex anomaly prior to cardiac catheterization, which may be difficult, prolonged, and of increased risk. REFERENCES
1. Okin JT, Vogel JHK, Pryor R, Blount SG Jr. Isolated right ventricular hypoplasia. Am J Cardiol 1969;24:135-40. 2. Enthoven R, Dunst M, Richman B. Congenital hypoplasia of the right ventricle and tricuspid valve with survival into adult life. Am J Cardiol 1963;11:532-6. 3. Sackner MA, Robinson MJ, Jamison WL, Lewis DH. Isolated right ventricular hypoplasia with atria1 septal defect or patent foramen ovale. Circulation 1961;24:1388-402. 4. Gallaher ME, Sperling DR, Gwinn JL, Meyer BW, Fyler DC. Functional drainage of the inferior vena cava into the left atrium-three cases. Am J Cardiol 1963:12:561-6. 5. Raghib G, Ruttenberg HD, Anderson RC, Amplatz K, Adams P, Edwards JE. Termination of left superior vena cava in left atrium, atria1 septal defect, and absence of coronary sinus. Circulation 1965;31:906-18. 6. Holtzman H, Lippman M, Nakhjavan F, Kimbel P. Postpneumonectomy interatrial right-to-left shunt. Thorax 1980; 35:307-g.
Myocardial tuberculosis presenting restrictive cardiomyopathy
as
H. K. Bali, MD, DM, S. Wahi, MD, B. K. Sharma, MD, I. S. Anand, DPhihOXON), B. N. Datta, MD, and P. L. Wahi, MD. Chandigarh, India From the Departments of Cardiology, Medicine and Pathology, Postgraduate Institute of Medical Education and Research. Reprint requests: Professor P. L. Wshi, Department of Cardiology, Postgraduate Institute of Medical Education and Research,Chandigarh 160 012, India. 414122043
120 3
lungs, with a normal ventilation pattern. A venogram demonstrated an extensive thrombus of the left leg veins extending into the popliteal vein with a patent left iliac vein. A follow-up echocardiogram 1 day later revealed persistently poor biventricular function and no right-sided masses. The patient’s cardiac rhythm ultimately returned to sinus and he was discharged on a regimen of warfarin sodium. One year after admission to the hospital, he was seen for routine follow-up and was found to be back to his baseline activity status. Our case illustrates the sudden, complete, and unpredictable embolization of free-floating thrombus within the right atrium. Lower extremity venography in our case demonstrated that the RATE was probably a single fragment of an even larger deep venous thrombus within the leg veins. The multiple defects seen on the lung perfusion scan may indicate that the visualized embolic event was only one of a series of subacute pulmonary emboli. It is surprising how well-tolerated the pulmonary embolus was, given the patient’s advanced left-sided heart disease. One can only speculate that chronic left ventricular disease may have prepared the right ventricle for variable degrees of increased pulmonary vascular resistance. Presumably, a deep venous thromboembolus will pass rapidly to the lungs if its size is small relative to the tricuspid apparatus. A previous case report by Arvan and DuBois5 documented a shower of multiple small emboli that appeared and persisted in the right atrium for several minutes before passing to the lungs. The thrombus in our patient’s right atrium was large and was present at the onset of imaging. Its chronicity is uncertain, but once the thrombus was across the tricuspid valve, it passed rapidly to the pulmonary circulation without fragmentation. The percentage of thromboemboli large enough to be held up within the right atrium is unknown. When echocardiography is performed prospectively in patients diagnosed with pulmonary embolism, RATE are infrequently found (incidence less than 1 %).6 However, prior to embolization, RATE are typically unsuspected, are clinically occult, and are often found on incidental echocardiography. Although the true frequency of RATE can never be known, the clinical implications of their detection in the ultrasound laboratory are quite clear. They are the echocardiographic harbinger of a large, often fatal, pulmonary embolus.‘? 4 This case report documents for the first time the actual passage of a single, large, unattached thrombus within the right atrium to the pulmonary circulation. The clinical course of this patient is notable for the unpredictability of embolization in this setting, the absence of symptoms noted following the visualized embolization, and the seeming e0icacy of anticoagulation after embolization without hemodynamic instability. REFERENCES
1. Kinney EL, Wright RJ. Efficacy of treatment of patients with echocardiographically detected right-sided heart thrombi: a meta-analysis. AM HEART J 1989;118:569-73. 2. Panidis IP, Kotler MN, Mintz GS, Ross J. Clinical and
Brief Communications
701
echocardiographic features of right atria1 masses. AM HEART J 1984;107:745-58. Saner HS, Asinger RW, Daniel JA, ElspergerKJ. Twodimensional echocardiographic detection of right-sided cardiac intracavitary thromboembolus with pulmonary embolism. J Am Co11 Cardiol 1984;4:1294-301. Farfel Z, Shechter M, Vered Z, Rath S, Goor D, Gafni J. Review of echocardiographically diagnosed right heart entrapment of pulmonary emboli-in-transit with emphasis on management. AM HEART J 1987;113:171-8. Arvan S, DuBois B. M-mode and two-dimensional echocardiography of pulmonary emboli. J Clin Ultrasound 1982;10:177-9. Kasper W, Meinertz T, Henkel B, Eissner D, Hahn K, Hofmann T, Zeiher A, Just H. Echocardiographic findings in patients with proved pulmonary embolism. AM HEART J 1986;112:1284-90.
Prolonged survival despite severe cyanosis in an adult with right ventricular hypoplasia and atrial septal defect Dean G. Karalis, MD, K. Chandrasekaran,MD, Mark F. Victor, MD, and Gary S. Mintz, MD. Philadelphia,
Pa.
Isolated right ventricular hypoplasia is an uncommoncardiac malformation. Both the right ventricle and tricuspid valve are underdeveloped. An interatrial communication, most often an atria1 septal defect, is universally present.’ Right-to-left shunting at the atria1 level develops, with normal pulmonary artery pressures.The clinical picture is dominated by right heart failure and cyanosis.The prognosisis generally poor and survival into adult life is rare.2 We report a caseof a 57-year-old man with severecyanosis due to a hypoplastic right ventricle with right-to-left shunting acrossan atria1 septal defect. To our knowledge, this is the oldest describedpatient to have such an anomaly. A 57-year-old man had clubbing and cyanosis since childhood. He had a long history of hypertension and polycythemia and had been receiving long-term hemodialysis therapy for 7 years. Several days prior to this admission, he developedprogressivedyspnea and wasevaluated at another hospital. He wasfound to be cyanotic and in severe congestiveheart failure. The arterial partial pressure of oxygen (POT) was 43 mm Hg on room air and did not appreciably increasewith 100% oxygen. Vigorous hemodialysis resulted in an improvement in pulmonary venous congestion;however, he remained cyanotic. A pulmonary perfusion scanshowedno abnormalities.Pulmonary artery From the Likoff Cardiovascular Institute, Department of Medicine, Hahnemann University Hospital. Reprint requests: K. Chandrasekaran, MD, Hahnemann University Hospital, Cardiac Ultrasound Laboratory, Broad and Vine Streets, Mail Stop 313, Philadelphia, PA 19102.
414122044
September
702
Brief Communications
American
Heart
1990 Journal
Fig. 1. Transesophagealechocardiographicfour-chamber view demonstrating an atria1septal defect (top arrow) and smallright ventricle (RV) and tricuspid valve (bottom arrow). Flow acrossthe atria1 septal defect was right to left. LA, Left atrium; LV, left ventricle; RA, right atrium.
pressurewas32112mm Hg and pulmonary capillary wedge pressurewas 14 mm Hg. He wastransferred to our hospital for further evaluation of refractory hypoxemia. There was central and peripheral cyanosis and finger clubbing. The heart rate was regular at 90 beats/min. The blood pressurewas 120/70 mm Hg. The lungs were clear to auscultation. The secondheart soundwassingleand there was a 2/6 holosystolic murmur at the left sternal border radiating to the apex. There wasno peripheral edema.Arterial blood gasdetermination on 100% oxygen revealed a pH of 7.52,a POZof 46, and a PCO~of 26. The hemoglobinwas15.1 mg/dl. The electrocardiogram demonstrated normal sinus rhythm and first-degree atrioventricular block. The QRS axis was a negative 60 degreesand there were new anterolateral ST and T wave abnormalities suggestive of ischemia. Chest roentgenogramrevealed a mildly enlarged cardiac silhouette and mild pulmonary edema. A transthoracic echocardiogram was performed. The study wastechnically inadequate; however, it revealed left ventricular hypertrophy and severeanterior wall hypokinesis.The right ventricle and tricuspid valve were not well visualized. A transesophagealechocardiogram was performed (Fig. 1). The right ventricle was small in size and the endocardial surface was smooth without trabeculations. The tricuspid valve anulus was small, nonstenotic, and measured 1.5 cm in diameter. These findings were consistent with right ventricular hypoplasia. The pulmonary trunk wasnormal. There wasa large secundumatria1 septal defect. Contrast transesophagealechocardiography demonstrated right-to-left shunting acrossthe atria1 septal defect. The patient underwent cardiac catheterization.
A flow-directed balloon catheter passedeasily through the atria1 septal defect and into the left atrium. The hemodynamic data are displayed in Table I. The shunt acrossthe atria1 septal defect was pure right-to-left. A right ventriculogram demonstrated a small, smooth hypokinetic right ventricle without trabeculations. The left ventriculogram demonstrated anteroapical hypokinesis and mild mitral regurgitation. The left ventricular ejection fraction was 35%. There wasa 70% stenosisover a long portion of the proximal left anterior descendingartery. The left circumflex and dominant right coronary arteries werenormal. The patient’s clinical condition improved; however, his cyanosispersisted. It wasfelt that his initial respiratory decompensationwasdue to pulmonary venous congestionfrom a recent anterior wall myocardial infarction. Since there was no evidencefor residual ischemia,his coronary diseasewas treated medically. In addition, since his cyanosiswaswell tolerated and sincehe had remained asymptomatic for his entire adult life, it waselected not to proceed with operative correction of his congenital heart disease.One year after this admissionhe remains asymptomatic. In adults, cyanosisresulting from shunt reversal at the atrial level rarely occurs with normal pulmonary artery pressures.However, an atria1 septal defect that is part of a complex congenital malformation may result in cyanosis without pulmonary hypertension because of obligatory right-to-left shunting acrossthe atria1 septum. In patients with hypoplasia of the right ventricle, survival is generally poor due to inadequate blood flow to the lungs, becauseof right heart failure, and from the consequencesof marked polycythemia. Survival into adulthood dependson the size
Volume Number
Table
120 3
Brief Communications 703
I. Cardiac catheterization Pressure (mm &?I (mean)
Site Superior vena cava Inferior vena cava Right atrium Right ventricle Pulmonary artery Right pulmonary artery Left pulmonary artery Left atrium Left ventricle Right lower pulmonary vein Left upper pulmonary vein Aorta
(12) 30112 30/14 (18)
02) 110/12 110/70
Oxygen saturation* (%) 64 52 60 59 56 55 62 80 19 96 96 80
*Obtained while patient was breathing 100% oxygen.
of the interatrial communication, the degreeof right ventricular hypoplasia, and the maintenance of adequate blood flow to the lungs.The age of onset of symptoms varies; however, prolonged survival into adult life is rare. Sackner et a1.3describeda patient who survived to the age of 30, dying from refractory right heart failure. The clinical course of our patient is unique due to his prolonged survival into adult life. Furthermore, despite marked cyanosishe has remained relatively asymptomatic. We assumethat this wasdue to multiple physiologic responsesthat preserved tissue oxygenation despite severe cyanosis,such assecondarypolycythemia and increasesin cellular 2,3-DPG (2,3-diphosphoglycerate). However, the greatly increasedhematocrit can lead to undesirableconsequences.In this patient depressederythropoiesis from chronic renal failure led to attenuation of the polycythemic response.In addition, the hypoplastic right ventricle protected the pulmonary vascularbed from increasedflow that occursin a simple atria1 septal defect, but fortuitously the degree of hypoplasia allowed sufficient flow, permitting adequate oxygenation and the prevention of right-sided heart failure. Moreover, it is possiblethat long-standing hypertension in our patient may have played a role in decreasingthe right-to-left shunt, thereby augmentingblood flow through the pulmonary artery. These mechanisms may explain the prolonged survival in our patient. The presenceof cyanosisin an adult with an atrial septal defect usually heralds the development of suprasystemic
pulmonary
hypertension.
Only rarely
has cyanosis
with an atrial septal defect been reported in the presence of normal pulmonary artery pressures.In the present case, cyanosisoccurred despite normal pulmonary artery pressures becauseof restriction of venous inflow by the hypoplastic right ventricle, causingan obligatory right-to-left shunt at the atria1 level. The tricuspid valve, though small, wasnot a significant abnormality; its size simply reflected hypoplasia of the right ventricle. Other anatomic anomalies may favor right-to-left shunting with normal pulmonary artery pressures.Gallaher et a1.4describedthree patients
with
abnormally
large
eustachian
valves
that
extended from the origin of the inferior vena cava to the
margin of the atria1 septal defect, selectively channeling blood into the left atrium. Similarly, congenital anomalous systemic venous connections that terminate in the atria1 wall at the site of the atria1 septal defect result in the return of venous blood into the left atrium.5 After pneumonectomy, cyanosisfrom shunt reversal acrossan atria1 septal defect may occur despite normal pulmonary artery pressures,possibly due to mechanical alterations in right atria1 and ventricular configurations.6 In conclusion,severalpoints in this caserequire specific attention. First, although this malformation is often corrected surgically, prolonged asymptomatic survival despite severe cyanosis may permit a conservative treatment strategy in some patients. Second, not all right-to-left shunting acrossan atria1 septal defect is associated-with severepulmonary hypertension. Although this congenital malformation is rare in an adult population, it and other anatomic anomaliesshould be consideredin the differential diagnosisof a cyanotic adult with an atria1 septal defect and normal pulmonary artery pressures.Finally, in evaluating a patient with congenital heart diseasein whom the transthoracic echocardiogramis not adequate, transesophagealechocardiography should be performed. This is especially important in a sick adult with a potentially complex anomaly prior to cardiac catheterization, which may be difficult, prolonged, and of increased risk. REFERENCES
1. Okin JT, Vogel JHK, Pryor R, Blount SG Jr. Isolated right ventricular hypoplasia. Am J Cardiol 1969;24:135-40. 2. Enthoven R, Dunst M, Richman B. Congenital hypoplasia of the right ventricle and tricuspid valve with survival into adult life. Am J Cardiol 1963;11:532-6. 3. Sackner MA, Robinson MJ, Jamison WL, Lewis DH. Isolated right ventricular hypoplasia with atria1 septal defect or patent foramen ovale. Circulation 1961;24:1388-402. 4. Gallaher ME, Sperling DR, Gwinn JL, Meyer BW, Fyler DC. Functional drainage of the inferior vena cava into the left atrium-three cases. Am J Cardiol 1963:12:561-6. 5. Raghib G, Ruttenberg HD, Anderson RC, Amplatz K, Adams P, Edwards JE. Termination of left superior vena cava in left atrium, atria1 septal defect, and absence of coronary sinus. Circulation 1965;31:906-18. 6. Holtzman H, Lippman M, Nakhjavan F, Kimbel P. Postpneumonectomy interatrial right-to-left shunt. Thorax 1980; 35:307-g.
Myocardial tuberculosis presenting restrictive cardiomyopathy
as
H. K. Bali, MD, DM, S. Wahi, MD, B. K. Sharma, MD, I. S. Anand, DPhihOXON), B. N. Datta, MD, and P. L. Wahi, MD. Chandigarh, India From the Departments of Cardiology, Medicine and Pathology, Postgraduate Institute of Medical Education and Research. Reprint requests: Professor P. L. Wshi, Department of Cardiology, Postgraduate Institute of Medical Education and Research,Chandigarh 160 012, India. 414122043