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+/-0.7 kg (2.5-4.3 kg)) had highly symptomatic TOF (Hb: 18+/-2g/dl, SO2: 68+/-11%); angiographic diameters: RPA: 4.1 mm (2.5-6.4 mm), LPA: 3.4 mm (1.6-7.0 mm), PA trunc: 4.4 mm (2.5-7.0 mm). 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+/-40 mm2/m2 (normal: 330+/-35 mm2/m2). Postpalliation angiograms (age: 10-14 months) demonstrated a significant catch-up growth in nine patients (PA index from 99+/-40 to 310+/-54 mm2/m2) and inadequate growth in two patients (PA index 63 and 115 mm2/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 (12-17 years) are in excellent clinical condition. Echocardiography revealed good RV function and near normal diameters at peak systolic pressures between 25 and 50 mmHg. 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.
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+/-0.7 kg (2.5-4.3 kg)) had highly symptomatic TOF (Hb: 18+/-2g/dl, SO2: 68+/-11%); angiographic diameters: RPA: 4.1 mm (2.5-6.4 mm), LPA: 3.4 mm (1.6-7.0 mm), PA trunc: 4.4 mm (2.5-7.0 mm). 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+/-40 mm2/m2 (normal: 330+/-35 mm2/m2). Postpalliation angiograms (age: 10-14 months) demonstrated a significant catch-up growth in nine patients (PA index from 99+/-40 to 310+/-54 mm2/m2) and inadequate growth in two patients (PA index 63 and 115 mm2/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 (12-17 years) are in excellent clinical condition. Echocardiography revealed good RV function and near normal diameters at peak systolic pressures between 25 and 50 mmHg. 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.
Authors: Mark A Law; Andrew C Glatz; Jennifer C Romano; Paul J Chai; Christopher E Mascio; Christopher J Petit; Courtney E McCracken; Michael S Kelleman; George T Nicholson; Jeffery J Meadows; Jeffrey D Zampi; Shabana Shahanavaz; Sarosh P Batlivala; Joelle Pettus; Amy L Pajk; Kristal M Hock; Bryan H Goldstein; Athar M Qureshi Journal: Pediatr Cardiol Date: 2022-04-05 Impact factor: 1.838
Authors: Chesney D Castleberry; Todd M Gudausky; Stuart Berger; James S Tweddell; Andrew N Pelech Journal: Pediatr Cardiol Date: 2013-10-06 Impact factor: 1.655
Authors: Paweł Dryżek; Tomasz Moszura; Sebastian Góreczny; Krzysztof W Michalak Journal: Postepy Kardiol Interwencyjnej Date: 2015-03-06 Impact factor: 1.426