The surgical repair of total anomalous pulmonary venous return (TAPVR) has evolved since the earliest corrective procedures were performed in the late 1950s. Here, we present the case of a patient who underwent repair of TAPVR as a child in 1960 at Texas Children's Hospital, and who was re-examined in 2007. We discuss the types of TAPVR and the various treatment approaches therefor.

In March 1960, a 6.3-kg, 14-month-old boy presented with findings typical of supracardiac TAPVR, the right and left pulmonary veins emptying into a common trunk. This trunk formed a vertical vein that drained into the left brachiocephalic vein, which in turn emptied into the superior vena cava. The child's medical history included a heart murmur at birth, perioral cyanosis during suckling, and failure to thrive. Surgery to correct the TAPVR was performed at Texas Children's Hospital by Denton A. Cooley, with assistance from John L. Ochsner.

The patient underwent a bilateral anterior thoracotomy. Dissection showed the ductus arteriosus to be a ligamentous structure. Cardiopulmonary bypass was instituted at a flow rate of 60 cc/(min·kg) for a total of 378 cc/min. A small patent foramen ovale (PFO) was observed. (It should be noted that patients who survive their 1st year of life with TAPVR almost always have a nonrestrictive atrial septal defect, rather than the restrictive PFO that was seen in this patient.) An incision was made in the common trunk of the pulmonary venous systems and in the posterior aspect of the left atrium. These structures were anastomosed by use of a 4–0 arterial silk running suture. The PFO was closed with 3 interrupted figure-8 stitches. The right atriotomy was then closed with 3–0 arterial silk suture. The patient tolerated the procedure well, received 100 cc of blood during the procedure, and was sent to the recovery room in good condition. The total pump time was 19 min, and the dependent pump time was 13.5 min. This surgical procedure was among the first of its kind.

Forty-seven years after having undergone repair of TAPVR, the patient (who was known by one of the authors) consented to be re-examined. Echocardiography showed no mitral valve regurgitation and no atrial septal defect. The patient's estimated right ventricular pressure, as measured by tricuspid regurgitation jet velocity, was 27 mmHg. No obstruction was seen at the surgical anastomosis site (Fig. 1), and the peak systolic pulmonary venous velocity was 54.3 cm/sec, within the normal range for a patient of this age¹ (Fig. 2). The patient's left ventricular ejection fraction was 0.55.

Fig. 1 Color-flow Doppler echocardiography (apical 4-chamber view) shows no obstruction at the surgical anastomosis site (arrows) of 47 years earlier.

Fig. 2 Pulse-wave Doppler echocardiography shows a peak systolic pulmonary venous velocity of 54.3 cm/sec.

A 24-hour Holter monitor captured data on heart-rate ranges, heart rhythms, and arrhythmias. The patient's highest heart rate was 149 beats/min. The minimum heart rate recorded during a 1-hour period was 55 beats/min at 2 AM, while the patient was asleep. There were 37 supraventricular ectopic beats, and no ventricular ectopy. The longest sinus pause lasted 1.4 sec and was followed by junctional escape rhythm (Fig. 3), which was the only notable abnormality. The patient, who was living a normal life, was judged to be in New York Heart Association functional class I.

Fig. 3 Electrocardiographic tracings from a 24-hour Holter monitor show junctional escape rhythm—the only notable abnormality 47 years after surgical repair.

The incidence of TAPVR has been reported as 0.008% of live births, with an approximate incidence of 2.2% in patients who have congenital heart disease.² Total anomalous pulmonary venous return is a congenital anomaly in which there is no connection between the pulmonary veins and the left atrium; the pulmonary veins instead connect directly into the right atrium or to a systemic vein by an alternative pathway.³ Because all venous blood returns to the right atrium, there is an obligatory connection between the left and right atria.³

In 1957, Darling and colleagues⁴ developed a classification system for this anomaly. Type-I TAPVR involves drainage into the systemic venous system via a left-side vertical vein (an embryologic remnant of the left cardinal system), which drains into the left brachiocephalic vein or directly into the right superior vena cava. Type I accounts for 46% of patients who have TAPVR.⁵ Type II, which accounts for 26% of such cases,⁵ involves drainage directly into the heart, usually via the coronary sinus or directly into the right atrium. Type III involves drainage via a descending vein that courses below the diaphragm and joins the inferior vena cava, hepatic veins, or portal system. This type, which typically features obstruction of the pulmonary venous return, is clinically apparent during the initial days of extrauterine life and accounts for 24% of patients who have TAPVR.⁵ Type IV, which is rare, involves multiple sites of drainage and encompasses approximately 5% of these cases.⁵

Most patients develop symptoms within the 1st year of life: tachypnea, feeding difficulties, frequent respiratory infections, and failure to thrive. Those patients who have pulmonary venous obstruction may present urgently within the 1st few days of life with tachypnea, cyanosis, and heart failure.⁶

The chest radiographs of patients who have unobstructed types of TAPVR typically exhibit right atrial and right ventricular hypertrophy with increased pulmonary blood flow. In patients whose return is to the left brachiocephalic vein, there may be a characteristic enlargement of the superior mediastinum, bilaterally in a figure-8 or snowman shape.⁶ An electrocardiogram will usually show right-axis deviation with right atrial P-wave abnormality and right ventricular hypertrophy.⁶

Accurate definition of the drainage sites is important in determining the appropriate surgical approach. Two-dimensional echocardiography has been shown to establish the diagnosis accurately.⁷ Catheterization may be necessary if echocardiography is inconclusive in determining the site(s) of the pulmonary venous connections. The anomalous pulmonary venous connection is usually seen readily after contrast material is injected into a pulmonary artery.⁶

The diagnosis of TAPVR is an indication for surgery. If the condition remains untreated, it is usually fatal before 1 year of age.³ As in the case of our patient, the very earliest procedures were performed through bilateral anterolateral thoracotomies with sternal division. Since 1963, the standard incision has been a median sternotomy.³ Thanks to better diagnostic capabilities and to improvements in surgical techniques, operative death has decreased from 15.8% in 1984³ to as low as 0 in a later case series.⁸ The repair of TAPVR has become a surgical procedure with very low risks of morbidity and death.

Regarding the long-term postoperative outlook, it is known that repair of supradiaphragmatic TAPVR in infants, children, and even adolescents is followed by a clinical course similar to that after the closure of a nonrestrictive ostium secundum atrial septal defect. Interestingly, our patient had experienced obstruction of the pulmonary venous flow, as was suggested by the perioral cyanosis in the child's medical history and as was evidenced by the restrictive PFO that was seen during surgery. Now that long-term survival is expected in most patients, the focus has turned toward the prevention of long-term morbidities, such as pulmonary vein stenosis and late arrhythmias. A 2002 study⁹ reported a postsurgical survival rate of 87.3% for patients at 18 years of age, with no difference according to anatomic type of TAPVR or surgical technique. Among the chief late complications is post-repair pulmonary venous obstruction, which occurs in 11% of patients and requires reintervention.¹⁰ Electrophysiologic studies have revealed evidence of sinus node dysfunction, including sinus bradycardia, sinus pauses, and chronotropic impairment in most of the patients 10 years postoperatively; however, significant atrial and ventricular arrhythmias appear to be uncommon.¹¹ Despite favorable outcomes overall, there has been no systematic, comprehensive, long-term evaluation of patients who underwent repair of TAPVR in early childhood.¹¹ In fact, in 2005, an outcome analysis of current management approaches in these patients¹⁰ recommended that such a study would prove valuable in evaluating the surgical techniques to correct TAPVR, and in evaluating the long-term outcomes.