Asian Cardiovasc Thorac Ann 1999;7:71-73
© 1999 Asia Publishing EXchange Pte Ltd
Anomalous Systemic and Pulmonary Venous Connections to Coronary Sinus
Soman Rema Krishna Manohar, MCh,
Jagan Mohan Tharakan, DM,1
Department of Cardiovascular & Thoracic Surgery India
1 Department of Cardiology Sree Chitra Tirunal Institute for Medical Sciences and Technology Thiruvananthapuram, India
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For reprint information contact: Soman Rema Krishna Manohar, MCh Tel: 91 471 44 3152 Fax: 91 471 44 6433 email: sctimst{at}renback.delhi.nic.in Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram 695011, India.
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Abstract
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A 3-year-old female child presented with total anomalous pulmonary venous connection to the coronary sinus coexisting with a persistent left superior vena cava. During the surgical correction, since there was no innominate vein, the left superior vena cava required diversion to avoid systemic desaturation. This was achieved by end-to-side left superior vena cava-to-left pulmonary artery anastomosis that was technically easier than transposing it to the right atrium.
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Introduction
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The coronary sinus is the most common site of drainage in the cardiac type of total anomalous pulmonary venous connection. The coronary sinus is also the usual termination of a persistent left superior vena cava. We report a rare case in which both of these anomalous veins drained into a large coronary sinus.
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Case Report
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A 3-year-old female child presented with a history of recurrent respiratory infections and delayed developmental milestones. Examination revealed mild central cyanosis, right parasternal heave, a wide fixed-split second heart sound, and a grade 2/6 ejection systolic murmur at the left sternal border. Chest radiography showed dextrocardia with a cardiothoracic ratio of 55%. Echocardiography revealed a normal atrial situs with dextroversion, all four pulmonary veins draining into a large coronary sinus, a secundum atrial septal defect, and mild pulmonary hypertension. At cardiac catheterization, the left superior vena cava was entered from the coronary sinus (Figure 1
). The right ventricular pressure (35/0 mm Hg) and the pulmonary artery pressures (38/18 mm Hg; mean, 18 mm Hg) were mildly elevated. Oxymetry values were suggestive of total anomalous pulmonary venous connection at the atrial level with a pulmonary vascular resistance index of 2.14 Wood units•m–2. A pulmonary arteriogram in the levo phase showed both left and right pulmonary veins draining into the coronary sinus and then into a large right atrium (Figure 2).
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Figure 1. Photograph showing the right heart catheter position. After coiling in the large right atrium, the catheter has entered the left superior vena cava through the coronary sinus.
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Figure 2. Left pulmonary arteriogram in the levo phase showing the left-sided pulmonary veins joining the coronary sinus and filing the large right atrium.
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During surgery, a central venous catheter could not be placed in the left superior vena cava due to technical problems before sternotomy. The left innominate vein was absent and there were bilateral superior venae cavae with the left vena cava smaller than that on the right. There was atrioventricular and ventriculoarterial concordance with dextroversion. The left superior vena cava and the common pulmonary venous chamber receiving all four pulmonary veins drained into a large coronary sinus. The atrial septal defect was 2 x 2 cm and of the fossa ovalis type. The left atrium was small.
Cardiopulmonary bypass was established with aortic, right superior vena caval, and inferior vena caval cannulation. The left superior vena cava and the left pulmonary artery were dissected while cooling the patient. At a rectal temperature of 20°C, the aorta was cross-clamped and cold cardioplegic arrest was achieved. On total bypass, the right atrium was opened and the coronary sinus was vented. Under low flows (0.5 L•min–1•m–2), the lower end of the left superior vena cava was divided between clamps and the upper end was anastomosed end-to-side to the left pulmonary artery that was side-clamped using 6/0 monofilament polyglyconate synthetic absorbable suture (Maxon; Davis & Geck, Danbury, CT, USA) as a continuous suture. The cardiac end of the left superior vena cava was ligated. After releasing the clamps, the bypass flow was increased and the patient was rewarmed. The septum between the coronary sinus and the fossa ovalis defect was excised and the roof of the coronary sinus was cut back to allow free drainage of the pulmonary venous blood into the left atrium. The interatrial communication, including the coronary sinus opening, was closed using an autologous pericardial patch. The child had a smooth postoperative recovery. Repeat echocardiography showed good correction of the anomalous pulmonary venous drainage and digital subtraction left superior venacavography showed good functioning of the left superior vena cava-to-left pulmonary artery anastomosis (Figure 3).
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Figure 3. Postoperative digital subtraction left superior venacavogram showing the functioning left superior vena cava-to-left pulmonary artery shunt and absence of the innominate vein.
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Discussion
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A persistent left superior vena cava coexisting with total anomalous pulmonary venous connection to the coronary sinus has been described.1 The usual surgical repair consists of cutting back the roof of the coronary sinus into the left atrium and closure of the atrial septal defect as well as the coronary sinus, directly or with a pericardial patch.2 The left superior vena cava-to-coronary sinus connection has to be disrupted to prevent severe systemic desaturation and this can be achieved by simple ligation of the left superior vena cava at its confluence with the innominate vein. In the absence of the innominate vein, a simple ligation of the left superior vena cava might produce elevation of the ipsilateral jugular venous pressure that can lead to facial edema, venous engorgement, and chylothorax.3 Since there was no central venous catheter, measurement of a trial occlusion pressure was not possible and therefore we decided not to ligate the left superior vena cava.
Our patient had only a mild elevation of pulmonary arterial pressure and pulmonary vascular resistance and a reduction in these values was expected postoperatively due to concomitant correction of the total anomalous pulmonary venous connection. Therefore, we decided to divert the left superior vena cava to the left pulmonary artery and the end-to-side anastomosis was technically easy as these structures were close together. Division and reimplantation of the left superior vena cava to the right pulmonary artery has been reported in a case of unroofed coronary sinus syndrome where other techniques of division such as an intra-atrial baffle or reimplantation to the right atrium were technically difficult.4 Reimplantation of the left superior vena cava to the right atrial appendage has been described.5 In our case, due to the dextroversion of the heart, the right atrial appendage was far away from the left superior vena cava and therefore reimplantation was considered to be technically difficult. Reimplantation to the right atrial appendage may be indicated in a similar combination of defects with associated pulmonary hypertension and elevated pulmonary vascular resistance.
The combination of anomalous systemic and pulmonary venous connections is usually seen in visceral heterotaxy syndrome. Bilateral bidirectional cavopulmonary shunt followed by subsequent Fontan completion by an extracardiac conduit is suggested in these patients in order to prevent pulmonary venous obstruction by an intracardiac baffle.6 At the 1-year follow-up, our patient was doing well. It is unlikely that this child's left lung will develop pulmonary arteriovenous malformation later.7 The left superior vena cava is relatively small with a larger amount of prograde flow from the right ventricle, which contains hepatic venous blood filling the main and right pulmonary artery, a situation that is equivalent to pulsatile bidirectional cavopulmonary anastomosis.
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References
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Abstract
Introduction
