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Anomalous venous connection of the superior vena cava to the left atrium
developed left ventricular hypertrophy without dilatation (Table I). At age 70, transient second-degree heart block was seen on electrocardiogram. The systemic blood pressure was 130190 mm Hg and the carotid upstroke was delayed. A harsh, grade 4/6 systolic precordial murmur that radiated to the neck was present. No precordial diastolic murmur was heard. Cardiac catheterization (Table I) now showed a 7S-mm Hg peak systolic pressure gradient across the aortic valve (Figure I). The aortic valve was replaced (August 1987) with a 23-mm Hancock porcine bioprosthesis. Seven months postoperatively, he is asymptomatic and active, with an ejection fraction by radionuclide angiography of 70% at rest. The excised native aortic valve consisted of 2 cusps weighing together 5.9 g (Figure 2). The posterior cusp was mobile but thickened by Jbrous tissue; it contained a single small deposit of calcium. The anterior cusp, in contrast, was immobile and contained heavy calcific deposits.
When calcium is deposited on a congenitally bicuspid valve, it usually is distributed relatively evenly on each of the 2 cusps.’ In the aforemen-
TABLE I Echocardiographic and Catheterization Data
Dimensions Lett ventricular wall (mm) Left ventricular cavity (ed/ps) Fractional shortening (%) Pressures (mm Hg) Right atrium (mean) Right ventricle (ps/ed) Pulmonary artery (ps/ed) Pulmonary artery wedge Left atrium (mean) Left ventricle (ps/ed) Aorta (ps/ed) Aorta to lett ventricle
(mm)
(mean)
peak systolic gradient Cineangiogram Aortic regurgitation Aortic valve calcium Cardiac output (liter/min) Cardiac index (liter/min/m*) Ed/ps = enddiastok/peak
Age 62
Age 70
12 60/35 42
18 53/35 34
7 30/10 28/12 12 12 155/13 155/60 0
7 32/8 28/15 10 210/17 135/70 75
3+/4 0 5.7 2.8
trace 3+/a+ 6.5 3.1
10
systole.
tioned patient, 1 cusp contained heavy deposits and the second was nearly devoid of calcific deposits. The reason for this uneven distribution of the calcific deposits is unclear. There was never clinical evidence of infective endocarditis. The cause of the valvular stenosis was the deposition on one of the cusps of extremely heavy calcific deposits that made this cusp, which was larger than the other cusp, completely immobile. The explanation for the deposition of heavy
Anomalous Venous Connection of the Superior Vena Cava to the Left Atrium
.
.
deposits of calcium on a prevtously purely regurgitant aortic valve is unclear, but such a documentation has not been recorded previously.
1. Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy casw. Am J Cardi 1970:26:72-83. 2. Roberts WC, Morrow
AG, McIntosh CL, Jones M, Epstein SE. Congenitally bicuspid aortic valve causing severe, pure aortic regurgitation without snperimposed infective endocarditis. Analysis of 13 patients requiring aortic valve replacement. Am J Cardial
1981;47:206-209,
was 95% and left atria1 saturation was 82%. Injections of contrast into the left atrium, the right pulmonary artery and the left pulmonary artery were unremarkable, demonstrating no apparent shunt. An WC injection demonstrated a large azygous vein connecting to the left atrium, con-
Michael A. Arsenian, MD, and Richard A. Anderson, MD bnormal drainage of caval blood into the left atrium represents a A rare cause of obligatory cyanosis.
notic but not clubbed. The heart sounds were distant and without murmurs or extra sounds. His heMost reported cases have presented moglobin was 20 g/d1 and his arteriat a young age in association with al PO2was46 torr. This increasedto other cardiac defects.‘v2 We report a 80 torr breathing 100% oxygen. The case of partial drainage of superior electrocardiogram showed left axis vena cava (SVC) blood into the left deviation with an intraventricular atrium in an asymptomatic, cyanotic conduction delay. His chest x-ray revealed a normal cardiac silhouette 74-year-old man. and normal lungfields. An echocarThe patient was hospitalized for kneesurgery andfound to becyanot- diogram showedmoderate right venic. He had a history of mild stable tricular enlargement and a nonspeexertional dyspnea attributed to cific septal wall motion abnormality. Right-sided cardiac pressures asthmatic bronchitis. He was cyawere normal at cardiac catheterizaFrom the Department of Medicine, Cardiotion. A catheter could be passed vascular Section, Maine Medical Center, from the right atrium into the left FIGURE 1. Superior vma cava i@ectb Portland, Maine. Manuscript received Februatrium through a patent foramen ehewlngerlglneflargeanemaleueveln ary 1, 1988; revised manuscript received and accepted June 27,1988. ovale. Pulmonary vein saturation onthl?left. THE AMERICAN
JOURNAL
OF CARDIOLOGY
NOVEMBER
1, 1988
989
right ventricular enlargement and hypertrophy are characteristically Abnormal systemic venous return lacking. A single Sz, due to a diminto the left side of the heart causes ished and early P2, has been decyanosis that, as in Eisenmenger’s scribecL2 Preexcitation occurs in a syndrome, cannot be corrected with high percentage of these patients.3 inhalation of 100% oxygen. Unlike The ultimate diagnosis of anomalous Eisenmenger’s however, signs of systemic venous drainage has required catheterization and angiotinuing below the diaphragm and entering into the splenic vein.
Innominate
Vefn
Anomalou Vl?lfl
graphic demonstration of abnormal flow, although contrast echocardiography may be a useful diagnostic modality once the entity is suspected.4 A left-sided SVC draining into the left atrium is the most common form of anomalous systemic venous connection to the left side of the heart. In most; though not all instances, there has been also a rightsided SVC draining into the right atrium. Abnormal drainage of the inferior vena cava into the left atrium has been reported, although it is considerably rarer. Other uncommon means by which such shunting may occur include drainage of an azygous vein, hemiazygous vein or coronary sinus into the left atrium. Direct communications between the SVC and a pulmonary vein may also occur. 1. Deleval MR, Ritter DG, McGoon DC, Danielson GK. Anomalous systemic venous connection. Mayo Clin
FIGURE 2. Catktehation data with preuurw and oxygen saturations. The cakubtd rlghtiloR ilow is 1.4. IVC = infariorvonacava;LA=leftatrium;LV = loft vqtrldq PA = pulmonary artery; PV=pdmowyvein;RA=tightptn’um;RV=rigMwnMcle;SVC=supen’or vona cava.
990
THE AMERICAN
JOURNAL
To Splenic
Vein ,r
R
flGURE 3. Posith of anomabus vein cowsingbehindtheheart.A=aorta. Othef abbrovlations as in Fire 2.
OF CARDIOLOGY
VOLUME
62
Proc 1975;50:599-610.
2. Perloff JK. Congenital vena caval to left atria1 communications. In: Perloff JK, ed. The Clinical Recognition of Congenital Heart Disease. Philadelphia:
WB Saunders,
1986:632-640.
3. James TN, Marshall TK, Edwards JE. Cardiac electrical instability in the presence of a left superior vena cava. Circulation 1976;54:689-697. 4. Snider AR, Ports TA, Silverman NH. Venous anomalies of the coronary sinus: detection by Mmode, twcFdimensiona1 and contrast echocardiography. Circulation 1978,60:721-727.
When calcium is deposited on a congenitally bicuspid valve, it usually is distributed relatively evenly on each of the 2 cusps.’ In the aforemen-
TABLE I Echocardiographic and Catheterization Data
Dimensions Lett ventricular wall (mm) Left ventricular cavity (ed/ps) Fractional shortening (%) Pressures (mm Hg) Right atrium (mean) Right ventricle (ps/ed) Pulmonary artery (ps/ed) Pulmonary artery wedge Left atrium (mean) Left ventricle (ps/ed) Aorta (ps/ed) Aorta to lett ventricle
(mm)
(mean)
peak systolic gradient Cineangiogram Aortic regurgitation Aortic valve calcium Cardiac output (liter/min) Cardiac index (liter/min/m*) Ed/ps = enddiastok/peak
Age 62
Age 70
12 60/35 42
18 53/35 34
7 30/10 28/12 12 12 155/13 155/60 0
7 32/8 28/15 10 210/17 135/70 75
3+/4 0 5.7 2.8
trace 3+/a+ 6.5 3.1
10
systole.
tioned patient, 1 cusp contained heavy deposits and the second was nearly devoid of calcific deposits. The reason for this uneven distribution of the calcific deposits is unclear. There was never clinical evidence of infective endocarditis. The cause of the valvular stenosis was the deposition on one of the cusps of extremely heavy calcific deposits that made this cusp, which was larger than the other cusp, completely immobile. The explanation for the deposition of heavy
Anomalous Venous Connection of the Superior Vena Cava to the Left Atrium
.
.
deposits of calcium on a prevtously purely regurgitant aortic valve is unclear, but such a documentation has not been recorded previously.
1. Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy casw. Am J Cardi 1970:26:72-83. 2. Roberts WC, Morrow
AG, McIntosh CL, Jones M, Epstein SE. Congenitally bicuspid aortic valve causing severe, pure aortic regurgitation without snperimposed infective endocarditis. Analysis of 13 patients requiring aortic valve replacement. Am J Cardial
1981;47:206-209,
was 95% and left atria1 saturation was 82%. Injections of contrast into the left atrium, the right pulmonary artery and the left pulmonary artery were unremarkable, demonstrating no apparent shunt. An WC injection demonstrated a large azygous vein connecting to the left atrium, con-
Michael A. Arsenian, MD, and Richard A. Anderson, MD bnormal drainage of caval blood into the left atrium represents a A rare cause of obligatory cyanosis.
notic but not clubbed. The heart sounds were distant and without murmurs or extra sounds. His heMost reported cases have presented moglobin was 20 g/d1 and his arteriat a young age in association with al PO2was46 torr. This increasedto other cardiac defects.‘v2 We report a 80 torr breathing 100% oxygen. The case of partial drainage of superior electrocardiogram showed left axis vena cava (SVC) blood into the left deviation with an intraventricular atrium in an asymptomatic, cyanotic conduction delay. His chest x-ray revealed a normal cardiac silhouette 74-year-old man. and normal lungfields. An echocarThe patient was hospitalized for kneesurgery andfound to becyanot- diogram showedmoderate right venic. He had a history of mild stable tricular enlargement and a nonspeexertional dyspnea attributed to cific septal wall motion abnormality. Right-sided cardiac pressures asthmatic bronchitis. He was cyawere normal at cardiac catheterizaFrom the Department of Medicine, Cardiotion. A catheter could be passed vascular Section, Maine Medical Center, from the right atrium into the left FIGURE 1. Superior vma cava i@ectb Portland, Maine. Manuscript received Februatrium through a patent foramen ehewlngerlglneflargeanemaleueveln ary 1, 1988; revised manuscript received and accepted June 27,1988. ovale. Pulmonary vein saturation onthl?left. THE AMERICAN
JOURNAL
OF CARDIOLOGY
NOVEMBER
1, 1988
989
right ventricular enlargement and hypertrophy are characteristically Abnormal systemic venous return lacking. A single Sz, due to a diminto the left side of the heart causes ished and early P2, has been decyanosis that, as in Eisenmenger’s scribecL2 Preexcitation occurs in a syndrome, cannot be corrected with high percentage of these patients.3 inhalation of 100% oxygen. Unlike The ultimate diagnosis of anomalous Eisenmenger’s however, signs of systemic venous drainage has required catheterization and angiotinuing below the diaphragm and entering into the splenic vein.
Innominate
Vefn
Anomalou Vl?lfl
graphic demonstration of abnormal flow, although contrast echocardiography may be a useful diagnostic modality once the entity is suspected.4 A left-sided SVC draining into the left atrium is the most common form of anomalous systemic venous connection to the left side of the heart. In most; though not all instances, there has been also a rightsided SVC draining into the right atrium. Abnormal drainage of the inferior vena cava into the left atrium has been reported, although it is considerably rarer. Other uncommon means by which such shunting may occur include drainage of an azygous vein, hemiazygous vein or coronary sinus into the left atrium. Direct communications between the SVC and a pulmonary vein may also occur. 1. Deleval MR, Ritter DG, McGoon DC, Danielson GK. Anomalous systemic venous connection. Mayo Clin
FIGURE 2. Catktehation data with preuurw and oxygen saturations. The cakubtd rlghtiloR ilow is 1.4. IVC = infariorvonacava;LA=leftatrium;LV = loft vqtrldq PA = pulmonary artery; PV=pdmowyvein;RA=tightptn’um;RV=rigMwnMcle;SVC=supen’or vona cava.
990
THE AMERICAN
JOURNAL
To Splenic
Vein ,r
R
flGURE 3. Posith of anomabus vein cowsingbehindtheheart.A=aorta. Othef abbrovlations as in Fire 2.
OF CARDIOLOGY
VOLUME
62
Proc 1975;50:599-610.
2. Perloff JK. Congenital vena caval to left atria1 communications. In: Perloff JK, ed. The Clinical Recognition of Congenital Heart Disease. Philadelphia:
WB Saunders,
1986:632-640.
3. James TN, Marshall TK, Edwards JE. Cardiac electrical instability in the presence of a left superior vena cava. Circulation 1976;54:689-697. 4. Snider AR, Ports TA, Silverman NH. Venous anomalies of the coronary sinus: detection by Mmode, twcFdimensiona1 and contrast echocardiography. Circulation 1978,60:721-727.