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Research Article

Early Outcome of Repair of Supra Cardiac Total Anomalous Pulmonary Venous Connection: Single Center Study

Faiz Rasool*

Assistant professor of pediatric cardiac/cardiovascular surgery, Children’s Hospital Lahore, Pakistan

Published Date: 05/05/2021.

*Corresponding author: Faiz Rasool, Assistant professor of pediatric cardiac/cardiovascular surgery, Children’s Hospital Lahore, 70 A 1 engineers town Lahore, Pakistan

DOI: 10.51931/OAJCS.2021.02.000026

Abstract

Introduction: The diagnosis of total anomalous pulmonary venous connection (TAPVC) is made when all four pulmonary veins drain anomalously to the right atrium or to a tributary of the systemic veins. It accounts for 1% to 1.5% of all congenital heart diseases. Supra cardiac TAPVC is the most common type of TAPVC that accounts for 45 to 55% of all TAPVCs. In this article we will discuss the results of supra cardiac TAPVC repair in our setup.

Patients and methods: It is a retrospective study. Files of all the patients who underwent supracardiac TAPVC repair at children’s hospital lahore in last 3 years (from January 2018 to December 2020) were reviewed. Age, weight, presenting symptoms, operative details (like cross clamp time and cardiopulmonary bypass time), peri operative mortality, ICU stay, and complications were studied.

Results: From January 2018 to December 2020, 48 patients under went repair of supra cardiac TAPVC at children’s hospital Lahore. Mean age was 13months, mean weight was 7kg, 9 (18%) patients died perioperatively. Ascending vertical vein was ligated in all cases, but in 2 patients we had to re-open the ascending vertical vein.

Conclusion: With only a few centers in Pakistan doing infant cardiac surgery, early outcome after repair of supra cardiac TAPVC is reasonable in our set up, but still far worse than that of modern centers. Good patient selection, and post-operative management of pulmonary hypertension can result in better outcome. In this series we did not present the follow up of the patients, which is drawback of this article.

Introduction

The diagnosis of total anomalous pulmonary venous connection (TAPVC) is made when all four pulmonary veins drain anomalously to the right atrium or to a tributary of the systemic veins. It accounts for 1% to 1.5% of all congenital heart diseases. It has 4 types (supracardiac, 45%; infracardiac, 25%; cardiac, 25%; mixed, 5%) [1]. The pulmonary veins establish an anomalous connection that delivers pulmonary venous blood to the right side of the heart instead of the left side. A patent foramen ovale or an atrial septal defect provides a right-to-left shunt so that a mixture of oxygenated and deoxygenated blood reaches the left side of the heart. Without the right-to-left shunting of blood, an infant would die [2]. Supra cardiac TAPVC is the most common type of TAPVC that accounts for 45 to 55% of all TAPVCs [3-6]. The connection in supracardiac TAPVC is usually to a left vertical vein draining into the left brachiocephalic vein through which pulmonary venous blood reaches right atrium.

Without surgical correction, TAPVC leads to greater than 78% mortality during the first year [7]. Surgical repair should be performed at the time of diagnosis, as an elective procedure. If surgery is delayed, the potential long-term complications associated with right heart volume and pressure overload can occur. The results for surgery for TAPVC have improved due to early referral, advanced surgical and anesthetic techniques, and better intraoperative and postoperative management [8]. However, repair does remain a challenge with early mortality reported in the literature in the range of <10% to 50%. Isolated TAPVC in a patient with biventricular (BV) anatomy, without severe concomitant cardiac defects, is associated with the best prognosis [9,10]. In this article we will discuss the results of supra cardiac TAPVC repair in our setup.

Patients and Methods

It is a retrospective study. Files of all the patients who underwent supracardiac TAPVC repair at children’s hospital lahore in last 3 years (from January 2018 to December 2020) were reviewed. Age, weight, presenting symptoms, operative details (like cross clamp time and cardiopulmonary bypass time), peri operative mortality, ICU stay, and complications were studied. Pre-operative evaluation: all the patients were admitted through OPD or emergency to cardiology/cardiac surgery ward. they were discussed in the cath conference. Decisions to operate were made after reviewing the echo cardiogram. Where in doubt, CT Angio was performed to confirm the diagnosis. Patients with single ventricle were excluded from the study

Surgical strategy

Patients were taken to the operation room after taking written informed consent. Standard anesthesia was given. Under sterile conditions, median sternotomy was made. Patients were put on cardiopulmonary bypass with standard aorto bicaval cannulation. In most of the patient’s temperature was cooled to 28 degree centigrade. 2 patients required deep hypothermic circulatory arrest. After clamping the aorta and DelNido cardioplegia in the aortic root, TAPVC was repaired with standard bi atrial approach. In 5 patients, sutureless technique [11] was used. In 3 patients’ superior approach [12] was used to repair TAPVC. Post operatively patients were shifted to cardiac surgical ICU on mechanical ventilation. Epinephrine and milrinone infusions were started intra operatively and were continued in post-operative period. No patient received nitric oxide. Operative mortality was defined as death within 30 days of an operation or within the primary hospitalization [13].

Results

From January 2018 to December 2020, 48 patients under went repair of supra cardiac TAPVC at children’s hospital Lahore.

Age: age of the patients ranged from 2 months to 12 years with the mean of 13 months.

Weight: weight ranged from 3.5 kg to 39 kg with the mean of 7 kg.

Cyanosis, dyspnea, failure to thrive and multiple respiratory infections were the chief presenting complaints.

Cardiopulmonary bypass time: 45 minutes to 241 minutes, with the mean of 81 minutes

Cross clamp time: 22 minutes to 184 minutes, with the mean of 62 minutes

Concomitant surgical procedures: atrial septal defect (ASD) was present in all cases, that was closed intra operatively. One patient had ventricular septal defect (VSD) that was closed concomitantly.

Ascending vertical vein was ligated in all cases but in 2 cases we had to re-open the vertical vein by removing the ligature. These 2 patients developed low cardiac output after coming off cardiopulmonary bypass but the hemodynamics improved when ascending vertical vein was re-opened.

Mortality: 9 patients died (18.75%). 8 out of 9 patients were having weight <5 kg. 3 patients died due to low cardiac output. 5 died because of persistent pulmonary hypertension, prolonged mechanical ventilation and multi organ failure. 1 died of post-operative bleeding.

Mechanical Ventilation: patients remained on mechanical ventilation for 6 hours to 15 days with the mean of 38 hours

ICU stay: average ICU stay was 70 hours (1 – 30 days).

Table 1: Demographical information.

Table 2: Surgical details.

Table 3: Gives the details of all the complications that occurred in post-operative period.

Discussion

Congenital heart disease (CHD) is the most common birth defect [14]. It is estimated that every year 42000 babies are born with CHD in Pakistan [15]. Children’s hospital Lahore is one of the largest pediatric cardiac surgery centers in Pakistan. We are doing > 1000 pediatric cardiac surgeries per year. In last 3 years we did 48 supra cardiac TAPVC repairs.

Mortality

Although there are few series which had mortality of 3 %[16,17] other centers reported that repair of TAPVC carried a relatively high early mortality (10–20 %) [18,19]. Postoperative care for TAPVC is based mostly on preventing or treating pulmonary hypertension and maintaining systemic cardiac output. Ideally, the pulmonary artery pressure will decrease to less than half of the systemic pressure soon after separating from cardiopulmonary bypass [20]. Those patients with severe pulmonary hypertension due to pulmonary venous obstruction are prone to develop reactive pulmonary hypertension. To treat pulmonary hypertension, patients are placed on nitric oxide and/ or a low dose of milrinone [21]. Administration of NO gas by inhalation has been shown to be beneficial to patients with PAH, particularly in paediatric cases [22-24]. pulmonary hypertension, pulmonary hypertensive crises and un availability of NO was major problems in those patients who died post operatively in our set up.

Keeping the vertical vein open

Whether the vertical vein should be ligated repair is controversial. We considered that it may be well to leave the vertical vein open if there is low cardiac output after coming off CPB. Keeping the vertical vein open can decrease the right ventricular load in the patient with serious pulmonary hypertension, and it can also alleviate the left ventricular load in the patient with poor ventricular compliance. Chowdhury et al. preferred that an adjustable means can be used to close up the vertical vein after operation [25]. Although vertical vein ligation is recommended to close shunting, small left heart chambers may not always tolerate the immediate increase in blood flow [26]. Transcatheter vertical vein closure might render patients with high LAP suitable candidates for further hybrid approach.

Risk factors

Weight less than 5 kg, pre-operative pulmonary venous obstruction, and persistent pulmonary hypertension were identified as risk factors.

Conclusion

With only a few centers in Pakistan doing infant cardiac surgery, early outcome after repair of supra cardiac TAPVC is reasonable in our set up, but still far worse than that of modern centers. Good patient selection, and post-operative management of pulmonary hypertension can result in better outcome. In this series we did not present the follow up of the patients, which is drawback of this article.

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