Case presentation:
A 7-day male neonate presented to our hospital with cyanosis, tachypnea
since birth.
Transthoracic Echocardiography (TTE) revealed mixed type TAPVC, small
patent foramen ovale (PFO), a large VSD (10 mm), a large patent ductus
arteriosus (PDA) with a right to left shunt, and severe pulmonary
hypertension (PH). For more anatomical details , computed tomographic
angiography (CTA) was performed, and confirmed the diagnosis of a mixed
type TAPVC (the common pulmonary confluent was draining both into the
coronary sinus and the left innominate vein through a vertical vein)
with an obstruction. The obstruction level was at the connection of the
vertical vein with the left innominate vein. On the 10th day of life,
the neonate developed a sudden cardiac arrest and underwent
cardiopulmonary resuscitation with rapid response without the need for
mechanical ventilation. The patient was scheduled for urgent surgical
repair. The operation was performed through median sternotomy. A large
vertical vein was seen on the left side outside the pericardium and
connecting with a very small vein to the left innominate vein
(representing the site of obstruction) (Figure 1). The vertical vein was
dissected and controlled. The pericardium was opened, and a large PDA
was controlled and closed by metal clips. Complete cardiopulmonary
bypass (CPB) was prepared with bicaval cannulation, the vertical vein
was closed at its junction with the left innominate vein, and the heart
was arrested by antegrade cold blood cardioplegic solution. The common
pulmonary confluent was seen behind the heart. The right atrium was
opened parallel to the right atrioventricular groove. A small PFO was
seen (representing another site of obstruction). The coronary sinus
orifice was cut through to unroof the coronary sinus and establish a
wide communication with the left atrial cavity after resecting the
atrial septum. A large fresh autologous pericardial patch was used to
baffle the created cavity toward the left atrial cavity. A large VSD was
closed by a bovine pericardium patch with interrupted sutures. The
remainder of the operation was completed uneventfully and the patient
was weaned off the CPB easily. The patient suffered from atrial
arrhythmias in the intensive care unit (ICU) on the second postoperative
day, and was managed appropriately. After one week on mechanical
ventilation, the patient was extubated, and on the
15th postoperative day was discharged from the ICU.
The patient was followed-up for six months and was in very good general
condition with significant improvement and weight gain.