pediatric-pathology-and-laboratory-medicine-2017

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Volume 7, Issue 1 (Suppl)

J Clin Exp Pathol

ISSN: 2161-0681 JCEP, an open access journal

Pediatric Pathology & Laboratory Medicine 2017

March 15-16, 2017

March 15-16, 2017 London, UK

International Conference on

Pediatric Pathology & Laboratory Medicine

J Clin Exp Pathol 2017, 7:1 (Suppl)

Unifocalization in patients with single and two-ventricle physiology

Eugen Sandica

Heart and Diabetes Center North Rhine-Westphalia, Germany

Aim:

This retrospective study reviews our results with unifocalization procedure of major aortopulmonary collateral arteries

(MAPCAS) in patients with single- and two-ventricle physiology.

Methods:

From August 2006 to September 2015, 15 patients with pulmonary atresia and MAPCAS have been operated at our

institution. Median age was 13 months with interquartile range (IQR) 0.13-109 and median weight 7.8 Kg (IQR 3.2-24), respectively.

In 11 patients, unifocalization was the first procedure, while in four patients this was done as a second procedure following modified

BT shunt implantation to a pulmonary artery branch, in two patients (in one of them together with correction of a total anomalous

pulmonary venous connection of supracardiac type) stent implantation and exploratory sternotomy was done. In three patients,

the intracardiac anatomy was not suitable for a two-ventricle correction. In all patients, the unifocalization of the MAPCAS has

been performed through a median sternotomy only. The unifocalization was performed concomitant with a modified BT shunt as a

pulmonary blood flow source in three patients with a Glenn procedure in two patients and with an intracardiac repair (closure of the

ventricular septal defect and right ventricle (RV) to pulmonary artery (PA) valved conduit) in 10 patients. A Glenn procedure and

two intracardiac repairs with RV-PA conduit have been performed, thereafter in the three patients with primary unifocalization and

shunt procedure. In three patients with unifocalization and intracardiac repair a fenestrated patch has been used for the ventricular

septal defect closure.

Results:

All patients survived the unifocalization procedure at a median follow-up of 57 months (IQR 12-121 months). Two patients

with single-ventricle physiology already received the Fontan palliation with a non-fenestrated extracardiac conduit. Four patients with

two-ventricle physiology needed catheter interventions for peripheral pulmonary artery stenosis and eventually stent implantation in

three cases. In the group of the patients with two-ventricle physiology after unifocalization and intracardiac repair, two patients had

an RV pressure estimate of one half systemic pressure, four patients had an RV pressure estimate of more than two thirds systemic

pressure while six patients had an RV pressure estimate of less than one third systemic pressure. All three patients with single-ventricle

physiology have good hemodynamics after unifocalization and bidirectional Glenn (one patient) as well as after unifocalization and

total cavopulmonary connection (two patients).

Conclusions:

In our experience, very good results can be obtained after unifocalization in patients with single- and two-ventricle

physiology. There is a need for conduit replacement due to growth of the patient or conduit degeneration as well as for catheter-based

interventions for peripheral pulmonary artery stenosis. Residual high pulmonary artery pressure and right ventricular dysfunction

remain of concern for long-term survival in this very difficult group of patients.