Ann Thorac Surg 2008;86:1684-1686. doi:10.1016/j.athoracsur.2008.04.091
© 2008 The Society of Thoracic Surgeons
Case Reports
Successful Surgical Correction of Total Anomalous Pulmonary Venous Drainage in the Sixth Decade
Amit Modi, MRCS,
Hunaid A. Vohra, MRCS, MD,
Ivan Brown, FRCR,
Stephen M. Langley, FRCS(CTh)*
Wessex Cardiothoracic Centre, Southampton University Hospitals NHS Trust, Southampton, United Kingdom
Accepted for publication April 25, 2008.
* Address correspondence to Dr Langley, Pediatric and Adult Cardiac Surgery, Pediatric Cardiac Surgical Research, Oregon Health and Science University, Doernbecher Children's Hospital, Portland, OR 97239 (Email: langleys{at}ohsu.edu).
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Abstract
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We report an unusual case of total anomalous pulmonary venous drainage (TAPVD), who was successfully treated by surgery at the age of 56 years. We believe that this is the oldest person in the English literature to undergo surgical correction of TAPVD. The pathophysiology and factors for prolonged survival are further discussed.
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Introduction
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Total anomalous pulmonary venous drainage (TAPVD) is rare, comprising of approximately 1.5% of all congenital heart abnormalities [1]. It is usually diagnosed in the neonatal period and is rarely seen in adults [2]. It requires surgical correction for survival as no catheter corrective treatment exists. In patients with TAPVD, all the venous blood returning from the lungs drains to the systemic veins, creating a large left-to-right shunt. Supply of oxygenated blood to the systemic circulation requires an atrial septal defect to allow oxygenated blood to enter the left ventricle. Without surgery, 80% babies with TAPVD die before their first birthday [3, 4]. We report a case of a 56-year-old woman who underwent successful surgical correction of TAPVD.
The patient complained of breathlessness at 7 years of age during a ballet lesson. According to the patient, investigations performed at another center revealed anomalous pulmonary venous drainage, but it remained unknown whether it was partial or total. Cardiac catheterization confirmed the diagnosis but failed to identify the exact drainage pattern, which was diagnosed at the age of 41 years by magnetic resonance imaging scan. She refused surgery at that age due to quoted high risk. Eventually her symptoms of breathlessness deteriorated, and she presented to us as a new patient to our department at the age of 56 years. On examination, she had minimal cyanosis and early clubbing. Oxygen saturations were 85% on air, regular pulse of 90 per minute, blood pressure of 142/64 mm Hg, and an ejection systolic murmur at upper left sternal edge. Blood tests revealed polycythaemia with hemoglobin of 182 gm/L and hematocrit of 51.5. An electrocardiogram demonstrated sinus rhythm with first degree heart block and a right bundle branch block. Chest roentgenogram revealed cardiomegaly with prominent superior vena cava and right heart enlargement. Transthoracic echocardiogram showed severely dilated right atrium and right ventricle, large atrial septal defect, and moderate tricuspid regurgitation (right ventricle systolic pressure = 90 mm Hg + right atrium pressure). The left ventricle was compressed by the right ventricle, but there was good left ventricle systolic function. The gradient across pulmonary valve was 50 mm Hg, the aortic arch was normal, and there was a pericardial effusion (< 2 cm) with no tamponade. A cardiac magnetic resonance imaging scan (Magneton Avanto; Siemens, Malvern, PA) was repeated, which demonstrated supracardiac type of TAPVD (Fig 1) and a vertical vein draining into the innominate vein (Fig 2). Cardiac catheterization revealed supracardiac TAPVD, moderate pulmonary stenosis (peak gradient of 30 mm Hg across the pulmonary valve), Qp:Qs = 2.2:1, large atrial septal defect (shunting right to left), and normal coronary vessels.
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Fig 1. Cardiac magnetic resonance imaging (sagittal view) showing vertical vein (dotted white arrow) draining into the enlarged innominate vein (solid white arrow).
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Fig 2. Cardiac magnetic resonance imaging (sagittal view) showing right and left pulmonary veins (dotted white arrows) opening into the confluence (solid white arrow). Also seen are the ascending aorta above the pulmonary trunk, which is next to the vertical vein (grey arrow).
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Surgical repair of TAPVD was carried out by a median sternotomy. The patient was placed on cardiopulmonary bypass by aorto-bi-caval cannulation and was cooled to 28° C. Antegrade cold blood cardioplegia was used. The left atrium was anastomosed to the pulmonary venous confluence and the atrial septal defect was partially closed leaving a small defect, and the vertical vein was ligated. The air was removed from the left heart and cardiopulmonary bypass was terminated. The cross-clamp time was 67 minutes and the total cardiopulmonary bypass time was 167 minutes. The patient was transferred to the intensive care unit in a stable condition on no inotropes. The patient made an excellent recovery and the patient was discharged on postoperative day 7. At follow-up, 2 years post-surgery, she is in New York Heart Association functional class I. The transthoracic echocardiogram showed normal left ventricle function, good right ventricle function, thickened pulmonary valve, mean gradient of 23 mm Hg, dilated right heart, moderate tricuspid regurgitation, pulmonary veins draining normally to the left atrium, and no intracardiac shunt.
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Comment
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The TAPVD results from failure of primordial pulmonary vein to unite with the lung plexus of the veins. Therefore, the pulmonary veins drain to the heart through systemic veins to the right atrium. In TAPVD, supply of oxygenated blood to the systemic circulation requires intracardiac communication between the right and left sides of the heart to allow oxygenated blood to enter the left ventricle. The size of the communication determines the volume of blood able to cross to the left heart, and therefore it determines the cardiac output and systemic oxygenation. In supracardiac TAPVD, the ascending vein usually unites with the innominate vein, which may drain directly to the superior vena cava or the azygous vein. In the infracardiac TAPVD, a descending vein drains either to the inferior vena cava, the portal vein, hepatic veins, or ductus venosus. In cardiac TAPVD, the anomalous veins drain into the coronary sinus or directly to the right atrium.
Patients with TAPVD usually present in the early neonatal period, often with profound cyanosis and shock, and they almost always require surgical treatment in the neonatal period [5, 6], often on an emergency basis. There are two anatomic factors that determine the patient's clinical status. First, the patient's cardiac output and supply of oxygenated blood is limited by the amount of blood that can cross the atrial septum. Therefore, the characteristics of the necessary right-to-left shunt determine systemic cardiac output and oxygenation. Second, an obstruction may occur in the path of the pulmonary venous drainage from the lungs to the systemic venous system. If obstruction occurs, egress of blood from the lungs is limited. The consequences of obstruction are limitation of pulmonary blood flow, pulmonary venous congestion, impairment of oxygenation, and elevation of pulmonary artery pressures. These events lead to life-threatening cyanosis in neonates. Increased pulmonary blood flow and pulmonary venous obstruction will result in pulmonary hypertension and eventually heart failure. Survival until the age of 56 is unusual, and increasing age is described as an unfavorable factor for successful surgical outcome. In this particular patient, survival to this age can be attributed to supracardiac type of TAPVD, an unobstructed pulmonary venous flow, a large intracardiac shunt to maintain the cardiac output, and a normal-sized left ventricle. Moreover, there were neither any associated anomalies nor any preoperative acidosis. Post-repair, there was no residual obstruction at the site of left atrial anastomosis, which could manifest as poor cardiac output and chest roentgenogram findings of pulmonary congestion. We believe that (on balance) the potential disadvantages of replacing the pulmonary valve (ie, increased length of surgery, long-term risks of valve complications, need for reoperation in the future) outweighed the advantages in a patient with a relatively small to moderate gradient across the valve. In fact, this decision probably proved to be correct as the patient is now 2 years postoperatively asymptomatic, with no increase in the gradient across the valve. Generally, long-term prognosis after successful repair of TAPVD is favorable. Approximately 10% to 15% of patients have evidence of late pulmonary vein obstruction, which tends to be recurrent and progressive. For this reason, long-term surveillance is important. Long-term follow-up showing freedom from events and death in patients undergoing surgery for TAPVD after 50 years of age remains to be determined.
In conclusion, this report describes a case of an unobstructed supracardiac TAPVD in a 56-year-old woman who was successfully treated by surgery. At this age, surgery should not be excluded as an option. However, careful perioperative assessment of this small group of patients is needed to exclude the presence of anatomical and physiological factors that could affect the outcome adversely. For surgery to be successful, a clear management plan should be drawn up for the preoperative, operative, and postoperative management of such patients in consultation with pediatric cardiologists, cardiac surgeons, anesthetists, and intensivists. Despite an excellent early outcome, late outcome is unknown.
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References
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- Ferencz C, Rubin JD, Loffredo CA, Magee CM. The epidemiology of congenital heart disease, the Baltimore-Washington infant study (1981–1989). Perspectives in pediatric cardiology, Vol 4. Mount Kisco, NY: Futura Publishing Co Inc; 1993.
- Correa-Villasenor A, Ferencz C, Boughman JA, Neill CA. Total anomalous pulmonary venous return: familial and environmental factors. The Baltimore-Washington Infant Study Group. Teratology 1991;44:415-428.[Medline]
- Gathman GE, Nadas AS. Total anomalous pulmonary venous connection: clinical and physiologic observations of 75 pediatric patients Circulation 1970;42:143-154.[Abstract/Free Full Text]
- Behrendt DM, Aberdeen E, Waterson DJ, Bonham-Carter RE. Total anomalous pulmonary venous drainage in infants. I. Clinical and hemodynamic findings, methods, and results of operation in 37 cases Circulation 1972;46:347-356.[Abstract/Free Full Text]
- Sano S, Brawn WJ, Mee RB. Total anomalous pulmonary venous drainage J Thorac Cardiovasc Surg 1989;97:886-892.[Abstract]
- Yalta K, Turgut OO, Yilmaz A. Asymptomatic total anomalous pulmonary venous connection with double drainage in a young adult: a case report. Heart Surg Forum 1007;10:E211–2.
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Abstract
Introduction
