Ann Thorac Surg 1996;62:566-568
© 1996 The Society of Thoracic Surgeons
Case Report
Absent Right Superior Vena Cava With Hypoplastic Proximal Inferior Vena Cava
H. Cem Alhan, MD,
lyas Kayacio
lu, MD,
Gül
ah Tayyareci, MD,
Cantürk Çakalaaolu, MD,
Mustafa diz, MD,
Sümer Tarcan, MD,
Besim Yiiter, MD
Siyami Ersek Thoracic and Cardiovascular Surgery Center, stanbul, Turkey
Accepted for publication February 21, 1996.
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Abstract
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We describe the precise preoperative diagnosis of a very rare anomaly that comprises absence of the right superior vena cava, persistence of the left superior vena cava, and proximal hypoplasia of the inferior vena cava draining into the left superior vena cava via the hemiazygos vein associated with a large atrial septal defect and tricuspid regurgitation.
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Introduction
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An absent right superior vena cava (RSVC) with a persistent left superior vena cava (LSVC) and hypoplasia of proximal inferior vena cava (IVC) is a rare variation of the anomalies of the major veins. This report describes the precise preoperative diagnosis of this particular type of anomaly, which comprises absence of the RSVC, persistence of the LSVC, proximal hypoplasia of the IVC draining into the LSVC via the hemiazygos vein, a large atrial septal defect (ASD), and tricuspid regurgitation.
A 17-year-old female patient presented with 3-year history of progressive shortness of breath, and fatigue. Physical examination showed anterior chest wall deformity, pulse rate of 80 beats/min, blood pressure of 110/80 mm Hg, and respiratory rate of 20/min. On auscultation, there was a grade 3/6 systolic murmur accentuated in the pulmonary area, and fixed splitting of the second heart sound. The electrocardiogram showed coronary sinus rhythm with an upright P wave in lead I and an inverted P wave in leads II and III. An incomplete right bundle-branch block pattern was also present. Chest roentgenography showed enlargement of the heart and increased pulmonary vascularity. The normal superior vena cava shadow was lacking. A Doppler echocardiogram showed right atrial and ventricular enlargement, a dilated coronary sinus, absent RSVC, a large secundum-type ASD, and severe tricuspid regurgitation.
Cardiac catheterization was performed simultaneously both from the right femoral and the basilic veins. From both the superior and the inferior approach the catheter was passed into the right atrium via the LSVC (Fig 1
). The course of the IVC was on the left side of the vertebral column. The relevant catheterization data indicated an atrial septal defect with a high degree of blood-mixing and 10 mm Hg pressure gradient across the pulmonary valve. Angiocardiograms showed situs solitus and concordant atrioventricular and ventricular arterial relationships. On magnetic resonance images, the proximal segment of the right IVC was hypoplastic, and blood from the lower part of the body was conveyed by a left-sided IVC. The left intraabdominal IVC was bifurcated into two branches: a large hemiazygos draining into the LSVC and a collateral to the distal portion of a right IVC (Fig 2). The right renal vein and the left hepatic vein were draining into the right IVC, and the left renal vein into the left IVC. The right hepatic vein was directly draining into the right atrium.
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Fig 1. . Catheters inserted from both the right basilic vein and the right femoral vein enter the persistent left superior vena cava.
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Fig 2. . Magnetic resonance image (A) and schematic diagram (B) showing the left intraabdominal inferior vena cava bifurcating into two branches: a large hemiazygos draining into the left superior vena cava (LSVC) and a collateral to the distal portion of a right inferior vena cava (RIVC). The right renal vein (RRV) drain into the right inferior vena cava, and the left renal vein (LRV) into the hemiazygos vein. The proximal portion of the right inferior vena cava (HRIVC) is hypoplastic. (ASD = atrial septal defect; CS = coronary sinus; RA = right atrium; RV = right ventricle; TV = tricuspid valve.)
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The patient was taken to operation with a diagnosis of absent RSVC, proximal hypoplasia of IVC, persistent LSVC, ASD, and tricuspid regurgitation. An atretic remnant of the RSVC was detected (Fig 3). The LSVC and right distal IVC were cannulated, and cardiopulmonary bypass was initiated. Intracardiac inspection showed a large secundum-type ASD (4 x 3 cm) and annular dilatation of the tricuspid valve. The ASD was closed with a pericardial patch, and De Vega annuloplasty was performed. The patient's recovery was uneventful. Ten months postoperatively, the patient is in excellent clinical condition with normal exercise tolerance.
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Comment
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An absent RSVC occurs in 1% of patients with persistent LSVC [1] and in 0.1% of children with congenital cardiac disease [2]. There have been fewer than 100 reported cases of absent RSVC in patients with situs solitus. The pathogenesis of absent RSVC and persistent LSVC remains undefined, but it likely occurs secondary to an aberrant flow pattern between the left and right anterior cardinal and innominate veins.
This malformation is often associated with other congenital heart defects, but without major hemodynamic significance. However, it may cause difficulties in venous catheterization or pacemaker electrode insertion [3]. It may also be associated with a normal cardiac anatomy [4] and is frequently discovered incidentally [5]. Associated dysrhythmias have been reported, possibly secondary to deformation of conduction pathways by coronary sinus dilatation [2].
In hemiabsence of the proximal IVC, part or all of the abdominal portion of the right IVC is absent. Blood from the lower part of the body is conveyed by a left-sided IVC, which is also named persistent left IVC. The left IVC may drain directly into the left atrium [6], which is exceedingly rare, or it may curve to the right at the level of the twelfth thoracic vertebra and communicate with the distal right IVC. In almost all cases of persistent LSVC associated with persistent left IVC, the anomalous IVC drains into the LSVC via the hemiazygos vein [7].
Absent RSVC with persistent left IVC is an extremely rare anomaly. It may be associated with other severe cardiovascular anomalies [8]. Modern imaging techniques such as spiral computed tomography and nuclear magnetic resonance imaging should be performed in any patient with suspected anomalous systemic venous connection to display the exact anatomic details.
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Footnotes
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Address reprint requests to Dr Alhan, Acibadem, 
ebboylu Sok, 2/8, Kadiköy, stanbul, Turkey 81010.
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References
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- Winter FS. Persistent left superior vena cava: survey of world literature and report of thirty additional cases. Angiology 1954;5:90–132.
- Hurwitt ES, Escher DJW, Citrin LI. Surgical correction of cyanosis due to entrance of left superior vena cava into the left auricle. Surgery 1955;38:903–14.
- Krayenbuhl CU, Lincoln JCR. Total anomalous systemic venous connection, common atrium, and partial atrioventricular canal. A case report of successful surgical correction. J Thorac Cardiovasc Surg 1977;73:686–9.[Medline]
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Abstract
Introduction
