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Eur J Cardiothorac Surg 2005;27:945-948
© 2005 Elsevier Science NL

Evidence for palliative enlargement of the right ventricular outflow tract in severe tetralogy of Fallot

^a Department of Thoracic- and Cardiovascular Surgery, University Hospital Duesseldorf, Duesseldorf, Germany
^b Department of Paediatric Cardiology, University Hospital Duesseldorf, Duesseldorf, Germany
^c Department of Paediatric Cardiology, Onassis Hospital, Athens, Greece
^d Research Group Experimental Surgery, University Hospital Duesseldorf, Duesseldorf, Germany

Received 8 October 2004; received in revised form 28 January 2005; accepted 7 February 2005.

^* Corresponding author. Address: Klinik für Thorax- und Kardiovaskuläre Chirurgie, Forschungsgruppe Experimentelle Chirurgie, Universitätsklinikum Düsseldorf, Moorenstr. 5, D-40225 Düsseldorf, Germany. Tel.: +49 211 811 9939; fax: +49 211 811 6996. (E-mail: schipke{at}med.uni-duesseldorf.de).

Objective: If the pulmonary artery (PA) tree in patients with Fallot's tetralogy (TOF) is extremely hypoplastic, a shunt procedure may be difficult and not desirable because of side-effects. Moreover, the failing catch-up growth of the pulmonary annulus is well known. In patients with a severe form of TOF, we performed palliative transannular patching of the right ventricular outflow tract. The early and long-term follow-up was evaluated. Methods: Eleven patients (93 days (10–245 days); 3.5±0.7kg (2.5–4.3kg)) had highly symptomatic TOF (Hb: 18±2g/dl, SO₂: 68±11%); angiographic diameters: RPA: 4.1mm (2.5–6.4mm), LPA: 3.4mm (1.6–7.0mm), PA trunc: 4.4mm (2.5–7.0mm). All 11 underwent transannular enlargement of the right ventricular outflow tract without closure of the ventricular septum defect. A PA index (cross-sectional area of the pulmonary arteries to BSA) was used to compare pre- and postoperative data. For follow-up, the patients were repetitively examined clinically and echocardiographically. Results: Preoperative PA index was 87±40mm²/m² (normal: 330±35mm²/m²). Postpalliation angiograms (age: 10–14 months) demonstrated a significant catch-up growth in nine patients (PA index from 99±40 to 310±54mm²/m²) and inadequate growth in two patients (PA index 63 and 115mm²/m²). Perioperative mortality was zero. Ten patients (43 months; 6–105 months) underwent elective repair. Six patients received pulmonary homograft valves (6–15 years after repair) because of severe pulmonary valve insufficiency and severe RV dilation. Complications: One patient died 10 months postpalliation due to pneumonia, one patient received a pacemaker after repair and died (2 months post-repair) due to pacemaker failure, a 5-year-old patient died 1 month after repair due to sepsis. All eight long-term survivors (12–17years) are in excellent clinical condition. Echocardiography revealed good RV function and near normal diameters at peak systolic pressures between 25 and 50mmHg. Only one patient developed brady-arrhythmia; a pacemaker was implanted 8 years after repair and 2 years after homograft implantation. Conclusions: In a very severe form of TOF, palliative right ventricular outflow tract construction may provide the potential for complete repair. In the presented high-risk patient group, mortality was not related to the hypoplastic pulmonary arteries. Obviously, all patients need pulmonary valve implantation in the long run.

Key Words: Tetralogy of Fallot • Palliation • Hypoplastic pulmonary artery • Enlargement of right ventricular outflow tract • Vascular catch-up growth • Pulmonary valve replacement

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