What is the Optimal Position for the Surgeon during an Infracardiac Total Anomalous Pulmonary Venous Drainage Repair?

In the video shown below, the authors describe the repair of an infracardiac total anomalous pulmonary venous drainage (TAPVD). The patient was born by ventouse delivery at term and in good condition; no resuscitation was required. The birth weight was 2.99 kg. At 12 hours of age, the patient was found to be hypothermic and hypoxic. He was intubated and ventilated for suspected persistent pulmonary hypertension of the new-born (PPHN). Due to increasing support and haemodynamic instability, poor oxygen index (OI), and nitric oxide inhalation (iNO), he was referred and accepted for mobile ECMO (extracorporeal membrane oxygenation) at two days of age.

VA (venous-arterial) ECMO support was established by the authors’ team in the referral hospital, via cervical cannulation. A 10 French Biomedicus arterial cannula was inserted into right carotid artery and a 12 French Biomedicus venous cannula was inserted into right internal jugular vein.

When the patient arrived at the authors’ department, a detailed echo demonstrated infracardiac TAPVD. The pulmonary venous confluence was very low and close to the diaphragm, positioned behind the ventricular mass rather than behind the atrium, as usually seen. A CT scan confirmed the diagnosis and demonstrated the obstruction, which was the cause of the pulmonary oedema and hypertension. These patients present early in the life with profound cyanosis and hypoxia.

The authors’ decided to support the patient on VA ECMO for 24 hours before attempting the surgical repair, to allow some lung recovery from the venous congestion and resuscitate the patient following the hypoxic insult.

Procedure

Cooling started on the ECMO circuit while the baby was transferred from the PICU. The chest was opened by the standard midline approach, a full dose of Heparin (100 u/kg) was administered, and ECMO was converted to cardiopulmonary bypass via the neck cannulae. Cooling was continued to 25ºc. The aorta was cross-clamped and the heart was arrested with cold blood antegrade cardioplegia. The ventricular mass was reflected into the right pleura, exposing the posterior pericardium.

At that point the surgeon moved to stand on the left side of the patient.

The posterior pericardium was incised and the descending vein was identified. This was ligated and transected. The confluence was then opened from an inferior to superior direction, and the pulmonary vein orifices were seen. The circulation was then arrested and the left atrial appendage was incised, and the incision was extended downwards into the body of the left atrium. The atrial communication was examined through this incision and was seen to be very large; it was therefore narrowed with a 7/0 Prolene suture. A direct anastomosis was then constructed between the left atriotomy and the incised confluence using 7/0 Prolene. The heart was placed back in the pericardium, the cross clamp was released, and the patient was re-warmed. Once the temperature had passed 32ºc, the bypass circuit was disconnected and the ECMO circuit was re-connected to the neck cannulae. Re-warming was continued, Heparin was partially reversed with 1mg aliquots of Protamine until the ACT was under 250 seconds, and blood products were administered. The sternum was closed and the baby was returned, on ECMO, to the PICU in good condition.

Bypass Data

The patient remained on ECMO for another 18 hours, maintaining a negative fluid balance and remaining on rest settings on the ventilator (20/10 rate 10, FiO2 40%). Then, a “retrograde flow” trial off was successfully obtained by adding a minimal amount of inotropes and reducing the centrifugal pump RPM to limit the amount of retrograde flow in the circuit.

During the trial off, the ventilation setting was increased to provide a 65% FiO2, with peak pressure of 25 cmH2O over a PEEP of 5 cmH2O and a ventilation rate of 30 breaths per minute. After satisfactory gas exchange and low pulmonary artery pressure was documented and stable for two hours, the patient was decannulated at the bedside. The neck vessels were reconstructed with 7/0 prolene continuous sutures and a central line was inserted into the right internal jugular vein using a semi-seldinger technique.

The patient was gradually weaned from the ventilator and then extubated on post-operative day three. The patient stayed two more days in the PICU and was discharged home on the 14th post-operative day.

References

Pump Controlled Retrograde Trial Off from VA-ECMO. Westrope C, Harvey C, Robinson S, Speggiorin S, Faulkner G, Peek GJ. ASAIO J. 2013 Sep-Oct;59(5):517-9. doi: 10.1097/MAT.0b013e31829f5e9f