Annals of Pediatric Cardiology
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Table of Contents   
Year : 2011  |  Volume : 4  |  Issue : 1  |  Page : 91-93
Selected Summaries

Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, India

Click here for correspondence address and email

Date of Web Publication15-Apr-2011

How to cite this article:
Talwar S, Choudhary SK, Airan B. Selected Summaries. Ann Pediatr Card 2011;4:91-3

How to cite this URL:
Talwar S, Choudhary SK, Airan B. Selected Summaries. Ann Pediatr Card [serial online] 2011 [cited 2021 Jan 20];4:91-3. Available from:

Hybrid Management Strategy for Percutaneous Fontan Completion Without Surgery : Early Results

Alsoufi B, Alfadley F, Al-Omrani A, Awan A, Al-Ahmadi M, Al-Fayyadh M, Al-Halees Z, Canver CC.

Ann Thorac Surg 2011;91:566-73.

This paper from King Faisal Heart Institute, Riyadh, is of interest because of the growing population of patients with single ventricle physiology in India. The authors of this paper report the early results of surgical preparation and subsequent percutaneous Fontan completion strategy in this subset of patients. Between 2002 and 2007, 277 patients underwent the bidirectional superior cavopulmonary anastomosis [BD Glenn (BDG)]. Thirty-four patients (median age was 7.7 months; range, 5 to 51 months; weight, >5 kg) had a lateral tunnel created at the time of BDG, which was fenestrated with 10- to 14-mm openings. The size of the fenestration is guided by the diameter of the inferior vena cava (IVC) at the level of the diaphragm on preoperative echocardiogram (10 mm if IVC <8 mm; 12 mm if IVC = 8-10 mm; and 14 mm if IVC >10 mm). The cardiac end of the superior vena cava (SVC) was patched from within the right atrium to maintain BDG physiology with mean bypass and aortic cross clamp times of 141 and 72 minutes, respectively. Median ventilation, intensive care and hospital stay durations were 1, 5 and 10 days, respectively. There was one early death due to persistently elevated pulmonary artery pressures; and two take-downs, one each due to elevated pulmonary artery pressures and recurrent effusions.

At the second stage, 25 patients underwent the percutaneous Fontan completion at a median age of 2.3 years (range, 1.5-4.2 years) and median interval after BDG of 16.4 months (range, 4.8-29.2 months). An angiogram was performed from the lateral tunnel as well as from the BDG to assess pulmonary artery anatomy, BDG, lateral tunnel and fenestration. The fenestration at the medial wall of the lateral tunnel was sized with a balloon, following which the autologous pericardial patch that separates the lateral tunnel from the right pulmonary artery (RPA) was perforated with radio-frequency energy. The communication of the lateral tunnel to the RPA was dilated with a percutaneous transluminal coronary angioplasty balloon, followed by a regular balloon. A proper-size Amplatzer atrial septal defect occlusion device was positioned across the fenestration but not released while dilatation of an SVC pericardial patch with the regular balloon was being performed. After the release of the Amplatzer device under transesophageal echocardiogram and fluoroscopy guidance, a stent mounted on a balloon was deployed between the upper part of the tunnel and the pulmonary artery. Finally, a tunnel angiogram was carried out to confirm a satisfactory result and the absence of any residual leak. Median procedure time was 195 minutes (range, 126-319 minutes), and median fluoroscopy time was 36 minutes (range, 23-81 minutes). In 3 patients, surgical Fontan completion was achieved because an additional intracardiac procedure was needed or because of issues with vascular access.

None of the patients who underwent percutaneous Fontan completion required inotropes, chest tube insertion or mechanical ventilation. Median intensive care and hospital stay durations were 1 and 6 days, respectively. There were no early deaths. Mean follow-up was 3.6 ± 1.5 years. There was one late death (unexplained cause), and one surgical revision was required due to obstruction of the systemic atrium by the bulky device. Five-year survival after BDG with Fontan preparation was 77% (range, 67%-87%), while 3-year survival after percutaneous Fontan preparation was 96% (range, 90%-99%).

The authors conclude that despite longer bypass and ischemic times, Fontan preparation at time of BDG is feasible and helps to achieve a future percutaneous Fontan completion that is associated with short recovery; low morbidity; and excellent early dynamics, and excellent echocardiographic and clinical outcomes.

Surgical Repair of Congenital Mitral Valve Malformations in Infancy and Childhood : A Single-Center 36-year Experience

Stellin G, Padalino MA, Vida VL, Boccuzzo G, Orru E, Biffanti R, Milanesi O, Mazzucco A

J Thorac Cardiovasc Surg 2010;140:1238-44.

This paper from the Pediatric and Congenital Cardiac Surgery Unit, University of Padua Medical School, Padua, Italy, is one of the largest experiences in this field, detailing results of surgical repair and predictors of the late outcome of congenital mitral valve dysplasia.

Between 1972 and 2008, 93 patients (43 males and 50 females) underwent mitral repair (median, 4.5 years; range, 0.16-19.8 years). Patients were divided into isolated (n= 32) and complex cases (n= 61) according to the complexity of associated lesions. Forty-eight (51.6%) patients had mitral regurgitation (MR) or predominant MR, whereas the remaining 45 (48.4%) had mitral stenosis (MS) or predominant MS. Associated cardiac anomalies were present in 67 (72%) patients. Surgical repair was tailored to the patient's valve anatomy and consisted of papillary muscle splitting (n= 35), cleft closure (n= 21), annuloplasty (n= 20), supravalvular ring excision (n= 16), commissurotomy (n= 15), chordal shortening (n= 9), ring annuloplasty (n= 6), commissuroplasty (n= 4) and accessory orifice closure (n= 2).

There were 7 (7.5%) early deaths due to low cardiac output syndrome (n= 4), hemorrhagic shock (n= 2) and mediastinitis (n= 1).

At a mean follow-up of 10.3 years (median, 8.4 years; completeness, 94%), there were 7 (8%) late deaths - due to congestive heart failure (n= 5), prosthetic valve thrombosis following mitral valve replacement (n= 1) and non-cardiac cause (n= 1). Sixty-five (81.25%) patients were in New York Heart Association class I or II. Sixteen (18.4%) patients required reoperations; these were on the mitral valve (MV) in 12 patients: 7 had residual MS, and 5 had residual MR. The mean interval between the first procedure and reoperation was 4.9 years (range, 2.2 months to 19.2 years). MV replacement was necessary in 11 patients, and re-repair was performed in one patient. Echocardiographic analysis at the time of last follow-up showed absent or minimal residual MV dysfunction in 30 (38.5%) patients, whereas 50 (62.5%) patients had hemodynamically significant residual valve dysfunction (residual MS in 28 patients and MR in the remaining 22 patients). The survival estimate for patients with MS was significantly lower than that for patients with MR. MS group was at higher risk for late heart failure (P= .026; OR, 3.870) and persistence of MV dysfunction (P= .049; OR, 2.545). On logistic regression analysis, MS was a significant risk factor for early operation (within the first year of life: OR, 7.219; 95% CI, 1.494-34.88) but not for early or late death (OR, 6.973; 95% CI, 0.0802-80.6). There was no significant difference between the isolated and complex groups in early need for surgical intervention or early and late mortality; however, the complex group had a significantly higher risk for presenting with unsatisfactory outcomes at follow-up (OR, 3.173; 95% CI, 1.158-8.693). Parachute MV was an important risk factor for late reoperation on the MV (OR, 6.817; 95% CI, 1.578-29.457), late heart failure (OR, 6.1; 95% CI, 1.309-28.420) and loss of sinus rhythm (OR, 6.377; 95% CI, 1.107-36.737). Also, residual stenosis of a parachute MV was a more common finding.

The authors conclude that mitral valve repair in infancy and childhood has acceptable rates of early mortality and reoperation. Mitral malformations in the complex group are associated with a significantly higher risk of reoperation, and parachute mitral valve is associated with a higher rate of early mortality.

Surgical Management of Congenital Heart Defects Associated with Heterotaxy Syndrome

Serraf A, Bensari N, Houyel L, Capderou A, Roussin R,

Lebret E, Ly M, Belli E

Eur J Cardiothorac Surg 2010;38:721-27.

Heterotaxy syndrome (HS) is associated with complex congenital cardiac defects, and there has always been a debate about referring such candidates for surgery. This study from Marie Lannelongue Hospital, France, answers this important question and details strategies to optimize the clinical outcomes.

Over a 20-year period, between 1989 and 2008, 139 patients with HS were offered surgery at the authors' institution. Fifty-one patients were found suitable for univentricular heart repair (UVR); and 88, for biventricular repair (BVR). Median age at first surgery was 4.4 months (range, 3 days to 43 years); 34 of these patients were neonates. Among those undergoing UVR,one patient had total cavopulmonary connection as the single and primary procedure; 12 had staged cavopulmonary connection with a bidirectional Glenn as initial procedure; and 23 had an initial palliation followed by bidirectional Glenn and Fontan completion. Two patients in the UVR group were switched to BVR. In the BVR group, 37 patients underwent primary repair; the remaining had a palliative operation. Overall, 62 patients had biventricular repair, 11 were switched to univentricular repair, six underwent a Fontan procedure and five patients had a bidirectional Glenn and were waiting for the final step. Of the whole group of patients, 34 required a neonatal procedure such as a Norwood operation, BT shunt, palliative arterial switch, co-arctation repair or pulmonary artery (PA) banding, depending on the indication. The mean number of surgical procedures per patient was 1.99. Reoperation was required in 22 patients - 15 after BVR and seven after UVR. Indications for reoperation in the BVR group were right ventricle to pulmonary artery (RV to PA) conduit replacement with PA plasty (n= 4), atrio-ventricular valve repair (n= 4), surgery for left ventricular outflow tract obstruction (n= 4), residual ventricular septal defect (n= 1), pace maker insertion (n= 1) and heart transplantation (n= 1). In the UVR group, after the final Fontan procedure, seven patients required re-interventions, for conversion to extra-cardiac conduit (n= 2), hepatic veins re-incorporation after Kawashima procedure with pulmonary arteriovenous fistulae (n= 2), pace maker insertion (n= 1), pulmonary veins stenosis (n= 1).

There were 23 (16.5%) early and six late deaths for an overall mortality of 20.8%. Eleven (7.2%) patients died after the very first surgery, six (6.6%) out of 90 died after the second surgical procedure and 6 (11.5%) out of 52 died after the third surgical procedure. In the UVR group, there were nine early and no late deaths for an overall mortality of 18%. In the BVR group, there were nine early deaths and one late death for an overall mortality rate of 15.6%. In the patients undergoing an initial palliation, there were five early and five late deaths for an overall mortality rate of 40%.

Median follow-up was 127 months (range, 1 month to 19 years). The overall survival at 15 years was 70.6%. It was 69% for the UVR group and 74.2% for the BVR group. Risk factors for overall mortality were neonatal surgery, longstanding palliation, total anomalous pulmonary venous return and right ventricular outflow tract obstruction. At the last visit, all survivors were in the New York Heart Association (NYHA) class I to II, and only two presented with supraventricular arrhythmias.

The authors conclude that HS remains a difficult situation, with high morbidity and mortality. An aggressive approach to repair total anomalous pulmonary venous return should be considered. Early decision to track the patient in either uni- or biventricular repair pathway should avoid long-lasting deleterious palliation. However, in the Indian context one should be cautious in generalizing these conclusions; and associated genetic defects, costs of surgery and the benefit accrued must be weighed.

Early Prophylactic Pulmonary Artery Banding in Isolated Congenitally Corrected Transposition of the Great Arteries

Metton A, Gaudin R, Ou P, Gerelli S, Mussa S, Sidi D, Vouhe P, Raisky O

Eur J Cardiothorac Surge 2010;38:728-34.

This study from University Descartes, Paris, France, presents the mid-term results of an aggressive surgical management strategy for isolated congenitally corrected transposition of the great arteries (ccTGA) by pulmonary artery banding (PAB) in early infancy. Between 2001 and 2009, 11 asymptomatic patients (seven neonates and four infants; mean age, 1.5 ± 1.4 months; mean weight, 4.1 ± 1.6 kg) with isolated ccTGA and intact ventricular septum underwent a dilatable, partially adjustable, homemade PA banding. PA banding was performed to maintain the condition of (rather than recondition) the left ventricle. PA band circumference was correlated to body weight (22 mm + 1 mm kg -1 ) and ideally adjusted to obtain flat septal geometry. Mean ventilation time and intensive care unit (ICU) stay were 20 ± 9 h and 2.6 ± 1.5 days, respectively. Five patients required postoperative inotropic support. There was no early death. There was one sudden late death at 4 months in a patient who had normal biventricular function and no tricuspid regurgitation. Mean follow-up was 21.5 ± 26 months. Mean band velocity increased over time - from 2.65 ± 0.7 m s -1 postoperatively to 3.7 ± 0.3 at 6 months and 4.5 ± 0.4 m s -1 at 2 years. Tricuspid regurgitation remained stable in seven patients, decreased in three and worsened in one. Flat septal geometry was obtained in all patients after the third postoperative month. One patient underwent an uneventful double-switch procedure at 7 years due to suprasystemic morphological-left ventricular pressure.

The authors conclude that in neonates with isolated ccTGA, prophylactic PA banding is safe and carries a low morbidity. At mid-term evaluation, tricuspid valve function is stabilized or improved, and systemic competence of the left ventricle is maintained, thus allowing a double-switch operation.

Correspondence Address:
Sachin Talwar
Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi -110029
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Source of Support: None, Conflict of Interest: None

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