Total Anomalous Pulmonary Venous Connection

Published on 07/06/2015 by admin

Filed under Neonatal - Perinatal Medicine

Last modified 07/06/2015

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19 Total Anomalous Pulmonary Venous Connection

I. CASE

A 29-year-old white woman, gravida 4, para 3, was referred at 31 weeks’ gestation by the high-risk obstetrician for abnormal triple screen with increased risk for trisomy 18 and an abnormal scan with mild disproportion at the ventricular level with increased right ventricular (RV) size. A small ventricular septal defect (VSD) was also suspected.

F. Neonatal management

1. Medical.

a. After birth, the baby will be assessed by the cardiac team. A low level of oxygenation (pulse oximeter <50% with low pressure and high oxygen ventilation) and progressive pulmonary hypertension and edema are the primary indications for intervention.

b. Management of the pulmonary hypertension includes assisted ventilation, although hyperventilation can also worsen the pulmonary edema and thus make oxygenation more difficult. For the most severe cases, an exit type of procedure with extracorporeal membrane oxygenation (ECMO) available for resuscitation may be necessary.

c. For total anomalous pulmonary venous connection (TAPVC) to the ductus venosus with obstruction, some have advocated the use of prostaglandin E1 (PGE1) infusion, but this is controversial.

d. Severely obstructed TAPVC requires emergency surgery because it cannot be medically managed.

2. Surgical.

a. Corrective repair consists of:

b. Nitric oxide might be helpful to treat associated persistent pulmonary hypertension, especially after surgery.