The neonate underwent an emergency open heart surgery by performing a side-to-side anastomosis between the pulmonary venous confluence and left atrium. The connection of the confluence with the SVC was ligated.
The postoperative recovery was complicated by pulmonary arterial hypertension, right ventricular myocardial dysfunction with hemodynamic failure, and unfortunately the newborn died 3 days after surgery.
Anomalous connection of the pulmonary venous confluence directly to the right SVC, especially the obstructed form is an unusual supracardiac variant. In a retrospective, collaborative study involving 19 pediatric cardiac centers from the United Kingdom, Ireland, and Sweden the incidence of TAPVC was about 7.1/100.000 live births. This incidence is higher compared to 6.8 in the Baltimore-Washington Infant Study or 5.9 in the New England Regional Infant Cardiac Program.[7–9] Several studies have reported case series with prenatally diagnosed supracardiac TAPVC,[10–12] some of them with drainage direct into the right SVC.[13,14] However, all of these cases were without obstruction at the drainage level. This is the first case of prenatally diagnosed isolated, obstructed supracardiac TAPVC with drainage directly into the right SVC, identified by us in the literature.
TAPVC is well tolerated in utero. This fact can be explained by the particularities of the fetal pulmonary circulation: low pulmonary blood flow, due to high pulmonary vascular resistance. However, Rasanen et al showed that during the pregnancy there is a slight increase of pulmonary blood flow compared with the combined cardiac output, from 13% at 20 weeks of gestation to 25% at 30 weeks of gestation, with no changes in the last semester. This pulmonary flow increase can explain the right-left ventricular discrepancy after 28 weeks of gestation. After postnatal transition, the pulmonary blood flow increases and as a result of systemic and pulmonary venous flow mixing in the right heart, the neonate will be cyanotic. If obstruction of pulmonary venous flow is associated, then pulmonary venous congestion occurs. Untreated, this can become very quickly a life threatening condition.
TAPVC can also be diagnosed after birth, during infancy, or even later. Recently, it was described a case of a 4-month-old infant without any pathological history, admitted for perioral cyanosis and poor feeding, who was diagnosed with TAPVC, which was successfully repaired by surgical intervention. Zhang et al in their study showed that comparing with surgical or AngioCT results, the postnatal echocardiography had a sensitivity of 97.6% and a specificity of 99.9%. Unfortunately, even though we diagnosed our patient in utero, he died after surgery. On the other hand, a study performed on patients with mixed-type TAPVC, underlined the fact that the mortality during the first year after surgery is high, but those who survive past the 1st year, usually present a good prognosis, without sequelae. TAPVC can be associated with other cardiac malformations, such as in the case described by Ide et al about a neonate diagnosed with a single ventricle, right atrial isomerism, pulmonary atresia with major aortopulmonary collateral arteries, a small central pulmonary artery and a supracardiac TAPVC, who underwent an uneventful surgical repair. Even though, complications after surgical repair, such as pulmonary arterial hypertension usually appear in the first 6 months after surgery, this can appear also later on life, like in the case described by Martinez–Quintana about a teenager who developed pulmonary venous obstruction and severe secondary pulmonary arterial hypertension after a surgical intervention for TAPVC during infancy. In the case described above, severe pulmonary arterial hypertension appeared very quickly after the surgical intervention and led to the newborn's death on the 3rd day after surgery. Other rare cases involving TAPVC were also reported in the specialty literature, such as the association between tetralogy of Fallot with anomalous left coronary artery from pulmonary artery and totally anomalous pulmonary venous connection, or the coexistence of TAPVC and persistent left superior vena cava. According to Shi et al obstructed TAPVC was discovered in 25% of newborns, the mean surgical age were about 215 days of life with 51 deaths of the 768 cases with different types of anomalous pulmonary venous connection.
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