Annals of Pediatric Cardiology
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Table of Contents   
Year : 2012  |  Volume : 5  |  Issue : 1  |  Page : 100-102
Selected Summaries

1 Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
2 Department of Cardiothoracic and Vascular Surgery, MIOT Hospital, Chennai, India

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Date of Web Publication12-Mar-2012

How to cite this article:
Talwar S, Singhi A. Selected Summaries. Ann Pediatr Card 2012;5:100-2

How to cite this URL:
Talwar S, Singhi A. Selected Summaries. Ann Pediatr Card [serial online] 2012 [cited 2022 Aug 17];5:100-2. Available from:

Should we always plan a fontan completion after a Kawashima procedure?

Setyapranata S, Brizard CP, Konstantinov IE, Iyengar A, Cheung M, d'Udekem Y

Eur J Cardiothorac Surg 2011;40:1011-15.

The Kawashima operation has long been considered a definitive palliation in patients with univentricular physiology and interrupted inferior vena cava (IVC) draining into the azygous or the hemiazygous system. This operation consists of a bidirectional superior cavopulmonary anastomosis (BDG) on the side of the drainage of the IVC. This paper from the Royal Children's Hospital, Melbourne, addresses the issue of the late incidence of pulmonary arteriovenous malformations (AVM) in these patients. The development of these malformations has been attributed to the isolation of the lungs from the exposure to hepatic factors.

Between 1990 and 2006, 21 patients underwent a Kawashima procedure at a median age of 2.3 years (0.5-8 years). The underlying anatomy included atrioventricular septal defect (n = 14), double-outlet right ventricle (n = 13), pulmonary atresia (n = 3), pulmonary stenosis (n = 11), total anomalous pulmonary venous drainage (n = 4 ) and bilateral superior vena cavae (n = 14). After the Kawashima operation, three patients remained with restricted forward flow from their systemic ventricle to the pulmonary arteries. There was one hospital death after Kawashima due to low-output syndrome. Before hospital discharge, the 20 Kawashima survivors had a median systemic oxygen saturation of 88.5% (76-94%). Follow-up was complete in all but one patient. Median follow-up was 14.5 years (3.6-19.8 years).

Thirteen patients developed pulmonary AVM after a median of 4 years (2-9 years) after Kawashima operation. These malformations were bilateral in eight patients and unilateral in five patients (three in the left lung). Diagnosis of pulmonary AVM was made at a median age of 6.6 years (2.4-16.3 years) and at a median of 4 years (1.9-8.8 years) after the Kawashima operation. A total of 16 patients underwent Fontan completion, 12 for cyanosis related to pulmonary AVM and four for decreased exercise capacity. Only three patients were left without Fontan completion at 4, 9 and 13 years after Kawashima. One of the 13 patients who developed pulmonary AVM died 4 years after Kawashima operation after an urgent repeated atrioventricular valve replacement for valve thrombosis. The rest of the 12 patients underwent Fontan completion (nine extra-cardiac and three lateral tunnels) at a median of 5.5 years (2.0-17.5 years) after Kawashima and at a median age of 7.5 years (3.0-25.6 years). Four of the 11 Fontan survivors had post-Fontan cardiac catheterisation. Pulmonary AVM were still detected in three patients at 4 months and 2 and 5 years post-Fontan. In two of these patients, hepatic flow was demonstrated to be draining only to one side of the lung and the ipsilateral vascular malformations had regressed, while the malformations in the opposite lung persisted. These two patients underwent Fontan revision. Among the seven patients who did not develop pulmonary arteriovenous malformations after the Kawashima, four underwent Fontan operation at 1.5, 2.2, 4.3 and 5.5 years after Kawashima operation. Freedom from development of AVM was only 47% at 5 years (95% CI: 23-69%). The authors concluded that unless some hepatic blood flow is directed to both lungs, most, if not all patients with a Kawashima procedure, will ultimately develop pulmonary AVM, and they recommend elective non-fenestrated Fontan completion in the years following Kawashima procedure.

Prophylactic amiodarone reduces junctional ectopic tachycardia after tetralogy of fallot repair

Imamura M, Dossey AM, Garcia X, Shinkawa T, Jaquiss RD

J Thorac Cardiovasc Surg 2012;143:152-6.

Junctional ectopic tachycardia (JET) is a common problem following pediatric heart surgery. After tetralogy of Fallot (TOF) repair, the incidence of JET may be as high as 15-20%, and this significantly prolongs the intensive care unit stay in these patients. In this paper from Arkansas Children's Hospital, the authors studied the role of prophylactic amiodarone in preventing JET after TOF repair.

Between November 2005 and November 2009, 63 consecutive patients underwent primary repair of TOF, of whom 20 had prophylactic amiodarone (amiodarone group) and 43 did not (control group). At the time of rewarming in the operating room while the patients were still on cardiopulmonary bypass, a continuous infusion of amiodarone was started at a rate of 2 mg/kg/d and continued for 48 h in the amiodarone group. Variables studied included demographic and bypass data, surgical procedure details (transannular or non-transannular patch), pre-operative and post-operative echocardiography findings and post-operative inotropic support. Univariate and stepwise multivariate analyses were conducted to determine factors associated with the occurrence of JET.

Both the groups were similar in age (5.1 ± 7.0 versus 2.7 ± 1.8 months), weight (5.9 ± 2.3 versus 5.3 ± 1.5 kg), bypass time (120 ± 28 versus 133 ± 44 min), rate of transannular patch usage (35 [81%] versus 18 [90%]) and pre-operative and post-operative gradient (84 ± 22 versus 84 ± 25 mmHg) through the right ventricular outflow tract in the control and amiodarone groups, respectively. The incidence of JET was 37% (16/43) in the control group and 10% (2/20) in the amiodarone group. Prophylactic amiodarone was significantly negatively associated with JET by both univariate (P = 0.039) and multivariate (P = 0.027) analyses. There were no adverse events attributable to prophylactic amiodarone use. The authors conclude that prophylactic amiodarone is well tolerated and significantly associated with a decreased incidence of JET after TOF repair. However, being a retrospective analysis, the results of this study must be interpreted with caution and need to be addressed by a prospective randomized trial.

Comparison of surgical, stent and balloon angioplasty treatment of native coarctation of the aorta: An observational study by the CCISC (congenital cardiovascular interventional study consortium)

Forbes TJ, Kim DW, Du W, Turner DR, Holzer R, Amin Z, et al; CCISC Investigators

J Am Coll Cardiol 2011;58:2664-74.

The appropriate management strategy for native coarctation of the aorta (CoA) is a matter for discussion. In the older age group, dilatation followed by stent placement is often preferred, and those patients who are younger often undergo surgery or a balloon dilatation (BA). Because of a lack of a prospective randomized study, it is difficult to determine the most appropriate form of therapy. In this prospective multi-institutional observational study about management of CoA in patients weighing >10 kg, the authors tried to shed some light on this issue by recruiting 350 native CoA patients form >30 institutions.

Between June 2002 and July 2009, 350 patients from 36 institutions were enrolled: 217 underwent stent placement, 61 underwent BA and 72 underwent surgery. Of the 72 undergoing surgery, 63% underwent end-to-end anastomosis repair, 17% tube graft interposition, 14% patch angioplasty and 6% subclavian flap repair. Two hundred and seventeen patients underwent 236 stent procedures. Nineteen patients required a second stent due to stent migration in six patients or to cover the entire CoA segment in the remaining patients. Age of the patients ranged from 1 to 76 years. Patients undergoing stent treatment were significantly older and weighed more than those undergoing BA or surgical treatment (P < 0.001). The authors found that patients in all the three arms showed significant improvement acutely and at follow-up in resting systolic blood pressure and upper to lower extremity systolic blood pressure gradient (ULG: upper lower limb gradient). When controlling for age and weight in the multivariate analyses, the stent group remained superior to BA in achieving a lower gradient (P = 0.008), with no differences observed between stent and surgery. The post-intervention catheterization systolic gradient from ascending to descending aorta was significantly higher in the BA group than in the stent group (P = 0.001), with multivariate analyses confirming the univariate results. When comparing the risk of all complications between the treatment arms while adjusting for confounders, stent was safer than BA (odds ratio [OR]: 5.72; 95% confidence interval: 1.59-20.52; P = 0.008) and surgery (OR: 11.23; 95% confidence interval: 3.66-34.51; P = 0.001). Surgical patients had a longer mean length of hospital stay as compared with stent patients. There were no early or late deaths. The mean follow-up was 1.9 years (range 0.1-5.8 years) in 68% of the stent patients, 2.1 years (range 0.07-9.09 years) in 77% of the BA patients and 1.9 years (range 0.1-9.7 years) in 81% of the surgical patients. Fifty-four patients had reinterventions (four in the surgery group, six in the BA group and 44 in the stent group) performed at a mean of 1.7 years after the initial procedure. These reinterventions were due to reobstruction, with no differences observed among the three groups. When examining short-term follow-up outcomes, the surgical group was superior in achieving a lower ULG compared with the BA group (P = 0.004). Stent and surgical repair continued to show a higher percentage of patients with normal BP when compared with BA patients. Short-term follow-up complication rates (both overall and aortic wall) remained lower in the stent group versus the BA group. No statistically significant differences in short-term follow-up complication rates were observed between the BA group versus the surgical group or between the stent group versus the surgical group.

The authors acknowledged that the study had limitations inherent to any non-randomized study, and concluded that the stent patients had significantly lower acute complications compared with surgery patients or BA patients, although they were more likely to require a planned reintervention. At shortand intermediate term follow-up, stent and surgical patients achieved superior hemodynamic and integrated aortic arch imaging outcomes compared with BA patients.

Anatomic repair for congenitally corrected transposition of the great arteries: A single-institution 19-year experience

Murtuza B, Barron DJ, Stumper O, Stickley J, Eaton D, Jones TJ, Brawn WJ

J Thorac Cardiovasc Surg 2011;142:1348-57.e1.

This paper from Birmingham Children's Hospital, Birmingham, is one of the largest single-institution experience with long-term follow-up of patients undergoing anatomic repair for congenitally corrected transposition of the great arteries (ccTGA) The authors focused on results in high-risk patients, the fate of the neo-aortic valve and occurrence of morphologically left ventricular dysfunction.

Between 1991 and March 2011, 113 patients (median age at repair 3.2 years range, 25 days to 40 years, weighing 3.2-61.4 kg) underwent anatomic repair for ccTGA. Double-switch (i.e., a Senning + arterial switch, DS) repair was performed in 68 patients, with Rastelli-Senning (RS)-type repair in 45. Pulmonary artery banding was performed for retraining the left ventricle in 23 patients. Median age at banding was 2.46 years (0.11-9.82 years), with median duration of banding of 562 days (5-2758 days), and five patients required a second banding procedure. Seventeen patients were classified as high-risk before anatomic repair, defined as requiring intensive care unit admission, ventilation and inotropic support pre-operatively. All had signs of severe congestive cardiac failure, six had pulmonary hypertension and six had severe tricuspid regurgitation (TR). An additional four patients had severe TR in the group requiring pulmonary artery banding.

Median cardiopulmonary bypass and cross-clamp times were similar in the DS and RS groups Patients were followed-up for survival status, morbidity and reinterventions.

There were five (of 68; 7.4%) early deaths in the DS group and 0 (of 45) deaths in the RS group. Actuarial survivals in the DS group were 87.6%, 83.9% and 83.9% at 1, 5 and 10 years versus 91.6%, 91.6% and 77.3% in the RS group (log-rank: P = 0.98). Freedom from death, transplantation or heart failure was significantly better in the RS group at 10 years (P = 0.03). There was no difference in reintervention at 10 years (DS, 50.3%; RS, 49.1%; P = 0.44). In the DS group, the Lecompte maneuver was associated with late reinterventions on the pulmonary arteries. Overall survival in the high-risk group was 70.6%. During follow-up, 14.2% patients had poor function of the morphological left ventricle, all in the DS group, but this was not related to pre-operative status or previous banding. Majority of the patients, after DS, had mild aortic incompetence, which appeared well tolerated. Annuloplasty of the aortic root at the time of DS reduced the risk of late aortic valve replacement.

The authors concluded that there is significant morbidity after anatomic repair of ccTGA, which is higher in the DS than in the RS group. Nevertheless, the majority of patients are free of heart failure at 10 years, including high-risk patients in severe heart failure before repair.

Correspondence Address:
Sachin Talwar
Additional Professor, Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

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