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Figure 3 Drawing of the heart. A, View of the anterior surface of the heart with
the anterior wall of the right ventricle (RV) opened to show the elongated anterior
tricuspid valve leaet (ATL) attached at the true anulus. Ao= aorta; AV= atrioventricular; IVC= inferior vena cava; PT= pulmonary trunk; RA= right atrium,
STL=septal tricuspid leaet; SVC=superior vena cava. B, View of the heart with
the anterior walls of the RA and RV removed to show the posterior and septal portions of the true anulus devoid of leaet attachments with the basal portions of the
STL and posterior tricuspid leaet (PTL) attached to the body of the RV. Caudal to
the true anulus and cephalad to the attachments of the STL and PTL is the atrialized portion of RV. Shown in the inset is a portion of the left ventricle (LV) and left
atrium (LA). The posterior leaet of the mitral valve prolapsed mildly into the LA.
VC-PFO=valvular competent patent foramen ovale. C, View of the right lateral surface of the heart showing the dilated RA and the posteriorly protruding atrialized
portion of RV. D, View of the heart with the right lateral wall removed showing the
basal attachment of the ATL to the true anulus (dashed line), but that of the PTL and
STL displaced caudally into the body of RV.
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Figure 4 Left anterior descending coronary artery at its site of maximal narrowing. Hemorrhage into the lipid portion of the plaque has occurred (Movat stain × 22).
Figure 5 Length of life and frequency of true atrial septal defect or patent foramen ovale (ASD) in 121 previously reported necropsy patients with Ebstein’s anomaly of the tricuspid valve.
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other congenital cardiovascular anomalies in these 121 necropsy patients. Of the 28
patients dying in the rst year of life, 26 (93percent) had an atrial septal defect or
patent foramen ovale; ve (18percent), a ventricular septal defect (in combination
with atrial septal defect or patent foramen ovale in four), and six (21percent), pulmonic valve atresia or stenosis. Of the 93 patients surviving the rst year of life, 76
(82percent) had an atrial septal defect or patent foramen ovale, and 17 (18percent)
did not; three (3percent) had a ventricular septal defect (in combination with atrial
septal defect or patent foramen ovale in two), and four (4percent) had pulmonic
valve atresia or stenosis. The mean age at death of those surviving the rst year
of life with an atrial septal defect or patent foramen ovale was 20years, whereas it
was 41years in those with an intact atrial septum (P<.001). Thus, although there are
exceptions, the presence of an atrial septal defect or patent foramen ovale overall
appears to adversely affect survival.
Death in our patient, as well as chest pain during the nal week of life, was
secondary to coronary arterial narrowing by atherosclerotic plaques rather than to
the congenitally abnormal tricuspid valve. To our knowledge, our patient is the rst
reported with Ebstein’s anomaly in whom death was attributed to atherosclerotic
coronary heart disease. Of 63 reported necropsy patients who survived past one
month of age and in whom the cause of death was determined, 16 (25percent) died
as a consequence of cardiac operation; 13 (21percent) from chronic congestive heart
failure; 12 (19percent), presumably from an arrhythmia because death was sudden;
six (10percent) from paradoxic embolus or brain abscess, ve (8percent), from complications of cardiac catheterization, and 11 (17percent) from noncardiac causes.
Thus, prolonged symptom-free survival with an anatomically severe form of
Ebstein’s anomaly is possible. The presence of an atrial septal defect or valvular
incompetent patent foramen ovale generally adversely affects survival.
REFERENCES
1. Adams JCL, Hudson R. Acase of Ebstein’s anomaly surviving to the age of 79. Br
Heart J 1956; 18:129–132.
2. Vacca JB, Bussmann DW, Mudd JG. Ebstein’s anomaly: complete review of 108
cases. Am J Cardiol 1958; 2:210–226.
3. Harris RHD. Ebstein’s anomaly: discovered in a 75-year-old subject in the dis-
secting laboratory. Can Med Assoc J 1960; 83:653–655.
4. Makous N, Vander Veer JB. Ebstein’s anomaly and life expectancy: report of a
survival to over age 79. Am J Cardiol 1966; 18:100–104.
5. Lev M, Liberthson RR, Joseph RH, Seten CE, Kunske RD, Eckner FAO, Miller RA.
The pathologic anatomy of Ebstein’s disease. Arch Path 1970; 90:334–343.
6. Seward JB, Tajik AJ, Feist DJ, Smith HC. Ebstein’s anomaly in an 85-year-old
man. Mayo Clin Proc 1979; 54:193–196.
7. Kumar AE, Fyler DC, Miettinen OS, Nadas AS. Ebstein’s anomaly: clinical prole
and natural history. Am J Cardiol 1971; 28:84–85.
8. Bialostozky D, Horwitz S, Espino-Vela J. Ebstein’s malformation of the tricuspid
valve: a review of 65 cases. Am J Cardiol 1972; 29:826–836.
9. Farooki ZQ, Henry JG, Green EW. Echocardiographic spectrum of Ebstein’s
anomaly of the tricuspid valve. Circulation 1976; 53:63–68.
10.
Giuliani ER, Fuster V, Brandenburg RO, Mair DD. Ebstein’s anomaly: the clinical
features and natural history of Ebstein’s anomaly of the tricuspid valve. Mayo
Clin Proc 1979; 54:163–173.
11.
Livesay WR. Clinical and physiologic studies in Ebstein’s malformation. Am
Heart J 1959; 57:701–711.
12.
Oldenburg FA, Nichol AD. Ebstein’s anomaly in the adult. Ann Intern Med 1960;
52:710–717.
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13. Genton E, Blount SG Jr. The spectrum of Ebstein’s anomaly. Am Heart J 1967;
73:395–425.
14.
Sekelj P, Benfey BG. Historical landmarks: Ebstein’s anomaly of the tricuspid
valve. Am Heart J 1974; 88:108–114.
15.
Hansen JF, Leth A, Dorph S, Wennevold A. The prognosis in Ebstein’s disease of
the heart: long-term follow-up of 22 patients. Acta Med Scand 1977; 201:331–335.
16.
Anderson KR, Lie JT. Pathologic anatomy of Ebstein’s anomaly of the heart revis-
ited. Am J Cardiol 1978; 41:739–745.
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Case 460 Total Anomalous Pulmonary Venous
Connection
Survival for 62Years Without Surgical Intervention
Bruce M. McManus, MD, PhD, Josef Luetzeler, MD, and
William C. Roberts, MD
Bethesda and Silver Spring, MD
Most patients with total anomalous pulmonary venous connection (TAPVC), irrespective of the drainage site, live for less than 6 months.
1, 2
Of reported nonoperated
necropsy patients with TAPVC with drainage of a common pulmonary vein into the
left innominate vein via a left vertical vein (“Snowman” type), none had survived as
long as 10years
necropsy verication was 39years of age.
1
(Figure 1). The oldest reported patient with TAPVC of any type with
1
Recently, we studied at necropsy a 62-yearold man with unoperated TAPVC with drainage of a retroatrial common vein into
the left innominate vein. He was cyanotic shortly after birth, acyanotic from about
age 20 to 40years, and cyanotic again during approximately his last 20years. He
was dyspneic on moderate exertion during his last 20years. He had an acute febrile
illness at age 48years characterized by cough and excessive dyspnea. At that time
chest roentgenogram disclosed cardiomegaly and a cavity in the left upper lobe.
The pulmonic second sound was increased in intensity. After antibiotic therapy the
signs suggestive of infection vanished. Because of cyanosis and cardiomegaly he
underwent cardiac catheterization 6 months later. Pressures (mm Hg) and oxygen
saturations (%) respectively were femoral artery (130/90, 78), left ventricle (110/3, 78),
right ventricle (94/5, 78), left atrium (5, 76), right atrium (6, 78); oxygen saturations
(%) superior vena cava (90), low left innominate vein (92), left subclavian vein (77),
left brachial vein (59); and cardiac output 5.6 L/min. The ECG at that time and 2 days
before death are shown in Figure 2. He never had overt congestive cardiac failure or
a precordial murmur. He died from complications of a perforated duodenal ulcer.
The ndings in the heart and lungs at necropsy are delineated in Figures 3 to 6.
Besides lack of excessive pulmonary vascular resistance, survival in TAPVC
appears to be dependent primarily on three factors: (1) the size of the defect in the
atrial septum; 2) the length of the anomalous pulmonary veins(s), and (3) the degree
of obstruction to ow in the anomalous pulmonary vein(s). Our patient appeared to
have survived so long because of nearly ideal characteristics of each of these three
factors. The atrial septum was virtually absent and therefore there was no interference to ow to the left side of the heart. The anomalous pulmonary vein was relatively short and free of any degree of obstruction. The cause of the total thrombotic
occlusion of the left main pulmonary artery in our patient was not determined. It is
clear, however, that this artery was totally occluded for at least 13years and maybe
considerably longer. Its occlusion in the presence of the left-to-right shunt (via the
TAPVC) may have further elevated the pulmonary arterial pressures.
3–7
From the Pathology Branch, National Heart, Lung and Blood Institute, National Institutes of
Health; and the Department of Pathology, Holy Cross Hospital of Silver Spring.
Received for publication Sept, 28, 1981; accepted Oct. 6, 1981.
186 DOI: 10.1201/9781003409342-26
Case 460 ToTal anomalous Pulmonary Venous ConneCTion
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Figure 1 Age at death or operation in 121 previously reported patients with isolated
TAPVC.
1, 2
Of those patients with the supracardiac “Snowman” type of TAPVC, 76%
(34 of 45) had died or been operated upon by age 1year, compared with 83% (63 of 76)
of those with other types of TAPVC. Only ve patients lived longer than 20years of
age and none of them had the “Snowman” type of pulmonary venous anomaly.
Figure 2 Electrocardiograms at age 48years, when the diagnosis of TAPVC was made
initially, and at age 62years, 2 days before death. On both occasions, sinus rhythm, right
axis deviation, right atrial abnormality, and right ventricular hypertrophy are present.
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Figure 3 Drawing of four-chamber view of the patient’s heart illustrating
the common atrium and anomalous pulmonary venous connection. Despite the
presence of calcic deposits, the congenitally bicuspid aortic valve appears to
have functioned normally. The anterior commissure of the aortic valve was not
in apposition with the posterior commissure of the pulmonic valve as is normally the case. The pulmonic valve is normal, as was the right ventricular outow tract.
Figure 4 Exterior (left) and interior (right) of heart. Ao = aorta; LA=left atrium;
LV=left ventricle; PT=pulmonary trunk; RA = right atrium; RV=right ventricle.
(Photographs by M.M.M. Moore)
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Figure 5 The right and left lungs demonstrating marked collapse and brosis of
the left lung and cavitation of the left upper lobe which contained Aspergillus species.
The left main pulmonary artery was completely occluded by thrombus. Extensive
thrombus also was present in the proximal branches of the right pulmonary artery
(arrows). The distal pulmonary arteries in the right lung are dilated.
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Figure 6 a, View of the tran sected aorta (Ao) at its isthmus and left mai n pulmonary
artery which is occluded by thrombus (T). Extensive calcic deposits are present in
the wall of the pulmonary artery. Lig=ligamentum arteriosum. b, Photomicrograph
of a small pulmonary artery in the left lung with multiluminal channels presumably
representing organized thrombus. Many such arteries were present. No plexiform
lesions were present. (Movat stain; original magnication ×18.)
REFERENCES
1. Burroughs JT, Edwards JE: Total anomalous pulmonary venous connection. Am
Heart J 59:913, 1960.
2. Delisle G, Ando M, Calder AL, Zuberbuhler JR, Rochenmacher S, Alday LE,
Mangini O, Van Praagh S, Van Praagh R: Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnostic and surgical
considerations. Am Heart J 91:99, 1976.
3. Harrison RW, Buehler WB, Thompson RG, Long ET, Carlson R, Charbon B, Adams
WE: Cardiopulmonary reserve ve to fteen years following 50percent or more
reduction of lung volume. Surg Forum 8:209, 1956.
4. Harrison RW, Adams WE, Beuhler WB, Long ET: Effects of acute and chronic
reduction of lung volumes on cardiopulmonary reserve. Arch Surg 75:546, 1957.
5. Harrison RW, Adams WE, Long ET, Burrows B, Reimann A: The clinical signi-
cance of cor pulmonale in the reduction of cardiopulmonary reserve following
extensive pulmonary resection. J Thorac Surg 36:352, 1958.
6. Rudolph AM, Neuhauser EBD, Golinko RJ, Auld PAM: Effects of pneumonec-
tomy on pulmonary circulation in adult and young animals. Circ Res 9:856, 1961.
7. Pool PE, Vogel JHK, Blount SG Jr: Congenital unilateral absence of a pulmonary
artery. The importance of pulmonary hypertension. Am J Cardiol 10:706, 1962.
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Case 479 FeTal rubella 27 years laTer
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Case 479 Fetal Rubella 27Years Later
Bruce F. Waller, MD, F.C.C.P., Frederick A. Smith, MD, Donald M.
Kerwin, MD, and William C. Roberts, MD, F.C.C.P.
Rubella infection in the rst trimester of pregnancy is now well recognized to
produce abnormalities in one or more body organs, including the cardiovascular
system, but few detailed descriptions of the morphologic alterations affecting this
system are available. Since 1963 at least six reports
diovascular ndings at autopsy in 45 patients born from women having had rubella
during early pregnancy: their ages in 37 were < one year; in four, one to ve years;
in three, six to ten years, and the oldest was 17years of age. The present report was
prompted by study at autopsy of an even older patient with typical features of the
“congenital rubella syndrome.”
A 27-year-old, severely mentally retarded woman who was born on Sept 14, 1952,
and died on March 12, 1980, had been asymptomatic all her life until 18hours before
death, when evidence of acute pneumonia developed, which proved fatal. At birth she had
been full-term but weighed only 2.2 kg. Aprecordial murmur was rst noted at age three
months. Her jaw protruded (prognathia), her teeth were malformed, her palate was arched,
and her left foot turned inward (valgus) when walking. Agrade 4/6 precordial systolic ejection-type murmur, with a thrill, was present, loudest along the upper left sternal border.
In the axillae and back, the murmur had both systolic and diastolic components. An ECG
showed right ventricular hypertrophy, and a chest roentgenogram disclosed a dilated pul-
monary trunk. Catheterization (Table1) disclosed multiple, severe (peak systolic pressure
gradient, 90mm Hg) peripheral pulmonary stenoses. She did well until the day of death,
when she began coughing, became rapidly dyspneic, febrile, hypotensive, hypoxic, and died.
The blood hematocrit was 55 percent; leukocyte count, 17,000/cu mm, and urinary protein
level, 3 + /4 +.
Injection of contrast material into the major extrapulmonary pulmonary arteries at
autopsy disclosed many discrete stenoses of the intrapulmonary pulmonary arteries (Figure1).
The pulmonary arteries proximal to the stenoses had severely thickened walls and the thickening resulted entirely from thickening of the media (Figure2). The right ventricular wall
was severely hypertrophied, and its cavity was not dilated (Figure3). The wall of the entire
aorta also was severely thickened, and again the thickening resulted entirely from thickening
of the media (Figures4 and 5). The wall of the left ventricle also was hypertrophied and its
cavity was not dilated (Figure3). The heart weighed 340 g. Histologically, the myocardium
was normal except for hypertrophy of the myocardial bers. Each kidney weighed about 100 g,
and histologically, hemosiderin deposits were present in the cytoplasm of many renal tubular
cells, indicating that intravascular hemolysis had occurred during life. The liver (1,200 g) and
1–6
have appeared describing car-
From the Pathology Branch, National Heart, Lung and Blood Institute, National Institutes
of Health, Bethesda, Md, and the Department of Pathology, Georgetown University Medical
Center, Washington, D.C.
Reprint requests: Dr. Roberts, Bldg 10A, Room 3E30, National Institutes of Health, Bethesda
20205
191DOI: 10.1201/9781003409342-27
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