Rare association of obstructed supracardiac total anomalous pulmonary venous connection with coarctation of aorta: case report

INTRODUCTION

Only 1.4% cases of TAPVC are reported to be associated with CoA.¹ In this patients lower body blood supply will be through saturated ductal flow. But if there is severe discrete coarctation with small closing PDA and obstructed supracardiac TAPVC, it forms bad subset.

CASE REPORT

Full term 2.4 kg newborn delivered by cesarean section admitted in NICU in view of respiratory distress and cyanosis. On examination baby was having tachycardia, tachypnea with distress and there was central cyanosis. Baby was kept on nasal CPAP with 60% FiO₂ but still not maintaining SpO₂ above 90%. Lower limb pulses were not very well palpable. X-ray showed significant pulmonary venous obstruction (Figure 1).

Echocardiography showed obstructed supracardiac TAPVC (all 4 pulmonary veins draining from common chamber to left vertical vein to innominate to right SVC to right atrium and the obstruction was at the level of left vertical vein), stretched PFO (R-L), severe discrete post ductal co-arctation with small PDA shunting R-L, dilated right atrium and right ventricle with moderate TR (max PG=60 mm of Hg) (Figure 2 and 3). Patient was intubated and started on PGE1 with dopamine. Baby was planned to shift for emergency TAPVC and coarctation repair on next day to nearby cardiovascular-thoracic surgery centre. Patient was having significant persistent metabolic derangements and lactic acidosis. Patient was having repeated episodes of bradycardia, desaturation inspite of full ventilatory and ionotropic supports. During such one episode baby couldn’t be revived and succumbed to death.

DISCUSSION

Association of coarctation have been rarely reported along with TAPVC. Only 1.4%, that is, 6 out of 422 patient have been previously reported.^1-3 Repair of infracardiac non obstructive TAPVC with CoA in 7 day old girl reported by Doksoz et al.¹ Leval et al reported a successful one stage repair of 12 day old male baby with diagnosis of non-obstructive supracardiac TAPVC with CoA.² Recently Hayashi et al reported successful repair of non-obstructive supracradiac TAPVC with coarctation in 6 day old boy.⁴ Generally in TAPVC case already there was low systemic output due to pulmonary venous obstruction and relatively small or restricted inter-atrial communication. But in our case there was obstructive supracardiac TAPVC along with severe discrete coarctation which lead to significant reduction in lower body blood supply and lead to rise in lactate levels and other metabolic de-arrangements, which caused very unstable haemodynamic status of baby inspite of high ionotropic and ventilator supports. We recommend urgent referral to CTVS centre of such baby for surgical treatment.

CONCLUSION

Obstructive TAPVC with severe coarctation patients are bad subset of cyanotic congenital heart diseases. They should be urgently referred to higher CTVS centre or if antenatally diagnosed such case should be delivered in such a centre which is well versed with such critical pediatric cardiac cases.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

REFERENCES

1. 1. Doksöz Ö, Güven B, Demirpençe S. Coarctation of the aorta with infracardiac total anomalous pulmonary venous drainage: a rare combination. Ann Thorac Cardiovasc Surg. 2014;20:778-80.
2. 2. de Leval MR, Stark J B-CR. Total anomalous pulmonary venous drainage to superior vena cava associated with preductal coarctation of aorta. Successful correction in a 12-day-old infant. Br Hear J. 1973;35:1098-03.
3. 3. Seale AN, Uemura H, Webber SA. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 2010;(122):2718-26.
4. 4. Hayashi H, Sughimoto K, Oka N, Tsuchida Y MK. Coarctation of the aorta with total anomalous pulmonary venous connection: a case report. AME Case Rep. 2021;5:11.