Annals of Pediatric Cardiology
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Table of Contents   
Year : 2013  |  Volume : 6  |  Issue : 2  |  Page : 207-210
Selected Summaries

Department of Cardiology, Vadamalayan Hospitals, Madurai, India

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Date of Web Publication20-Jul-2013

How to cite this article:
Kannan BR. Selected Summaries. Ann Pediatr Card 2013;6:207-10

How to cite this URL:
Kannan BR. Selected Summaries. Ann Pediatr Card [serial online] 2013 [cited 2019 Apr 25];6:207-10. Available from:

Outcome Predictors and Implications for Management of Scimitar Syndrome

Dusenbery SM, Geva T, Seale A, Valente AM, Zhou J, Sena L, et al.

Am Heart J 2013;165:770-7

Scimitar syndrome consists of unilateral anomalous pulmonary venous connection to the inferior vena cava, frequently associated with hypoplasia of the concordant lung and aortopulmonary collateral arteries to the hypoplastic lung. The goals of this study were to determine the risk factors for postoperative pulmonary vein stenosis or death and to identify the predictive factors for survival without scimitar vein surgery.

Between November 1964 and March 2011, 80 patients were identified. Two of them had left-sided Scimitar syndrome associated with heterotaxy and an interrupted inferior vena cava (IVC). More than half of the patients were diagnosed under the age of one year. Compared to the older group, these children were more likely to have aortopulmonary collaterals (APC), other cardiac anomalies excluding atrial septal defect (ASD), non-cardiac anomalies/syndromes, and cardiac or respiratory symptoms. There was a trend in the younger patients of having scimitar vein obstruction. There was no difference with regard to right and left ventricular size or systolic function, degree of left-to-right shunting or percent perfusion of the right lung. However, patients diagnosed in the first year of life were more likely to have pulmonary artery pressure greater than half of the systemic level. Fifty-one (64%) had all the pulmonary veins from the hypoplastic lung draining to the IVC. In 15 (19%), there were one or two small additional pulmonary veins from the hypoplastic lung connected to the left atrium, but had no connections to the pulmonary veins connected to the IVC. In eight (10%), anastomotic connections between the dual drainage were seen.

Fifty-six (70%) had APC, of which 31 had transcatheter coil occlusion, 12 underwent surgical ligation, 13 did not undergo any therapy, as they were small, and two had closed spontaneously. No patient who had a coil or a surgically closed APC developed pulmonary infarction. Recanalization occurred in one patient, with surgical ligation, and in three patients with coil occlusion, all were subsequently closed with coils. Scimitar vein obstruction was documented in 17 (21%), of which 13 were infants. Follow-up data was available in six patients. Transcatheter dilatation with cutting balloon was done in one patient who developed severe restenosis. The baffle procedure was performed in two patients; one developed severe restenosis, and mild persistent obstruction was seen in the other one. Reimplantation of the scimitar vein was performed on three patients and was associated with obstruction in two patients.

Sixty-one (76%) out of 80 patients survived during a median follow up of 6.2 years (0-42.2), 33 of whom had undergone scimitar vein surgery. The others had a baseline or post APC coil occlusion Qp: Qs of < 1.6. Following scimitar vein surgery, postoperative obstruction was documented in 18 patients. Thirteen of 28 patients with a baffle procedure and five of eight with reimplantation also showed pulmonary venous obstruction. There was a trend toward increased risk for postoperative pulmonary vein obstruction when scimitar vein surgery was performed in the first year. The univariate predictors of survival without scimitar vein intervention in this cohort were: Normal pulmonary artery pressure and absence of associated heart defects other than ASD. Of the 19 patients who died, additional heart defects were present in 15. Of the other four patients, one premature infant had a large intraventricular hemorrhage, two died after pneumonectomy, and one, who had pneumonectomy in infancy, died at the age of 40 years. The risk factors for death were age of presentation ≤ 1 year, additional heart defects other than ASD, non-cardiac anomalies/syndromes, pulmonary artery (PA) pressure greater than half-systemic, and left pulmonary vein stenosis.

In this study on Scimitar syndrome, normal PA pressure and absence of other congenital heart disease (CHD), excluding ASD, were factors predictive of survival without surgical intervention. Closure of APC did not cause pulmonary infarction, but it could reduce pulmonary artery pressure, and could potentially avoid the need for scimitar vein surgery. Surgical intervention was associated with a high rate of postoperative pulmonary vein obstruction, especially when performed in infancy. These observations may guide the management decisions in patients with scimitar syndrome.

Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center

Brescia ST, Rossano JW, Pignatelli R, Jefferies JL, Price JF, Decker JA, et al.

Circulation 2013;127:2202-8

Left ventricular noncompaction (LVNC) has been identified in association with a variety of congenital heart malformations and has been recognized in its isolated form as a distinct form of cardiomyopathy, accounting for nearly 9% of the newly diagnosed cardiomyopathies. Although this entity commonly presents with heart failure in infancy, a subset of patients develop symptoms in adolescence and adulthood. In addition, some individuals with characteristic findings of LVNC will never develop symptomatic heart failure. This retrospective review was aimed at examining the mortality profiles and the incidence of arrhythmias and sudden cardiac death in this patient population.

Two hundred and forty-two children diagnosed with having LVNC between January 1990 and January 2009, were studied, of whom 145 (60%) were males. The mean age at diagnosis was 7.2 ± 6.9 years, with 95 (39%) patients presenting in infancy. Forty-six (23%) patients had a family history of cardiomyopathy. The median duration of follow up was 4.0 years (1.8 - 15.9 years). Sixty (25%) patients presented with heart failure. Of the 42 (17%) patients presenting with arrhythmias, 25 presented with documented arrhythmia, five presented with aborted sudden death, and 12 were referred for evaluation of syncope. Nine percent presented with chest pain, 19% were referred for evaluation of murmurs, 10% were with abnormal baseline electrocardiogram (EKG) findings, and 7% were with cardiomegaly noted on a chest radiograph. Fourteen percent were detected to have LVNC on screening echocardiography or incidentally.

The patients were divided into four distinct phenotypes: Dilated (19%), hypertrophic (27%), mixed (62%), and normal dimensions (26%). Fifty-four percent had systolic dysfunction at presentation, 8% developed this during follow up, after an average of 47 months (1-175 months). Depressed systolic function (ejection fraction < 55%) was noted in 89, 42, 87, and 35%, with dilated, hypertrophic, mixed, and normal dimension phenotypes, respectively. Only 13% had normal EKGs; 41% had voltage criteria for ventricular hypertrophy. Other findings were diffuse T inversion (39%), ST segment abnormalities or strain (34%), atrial enlargement (19%), and left axis deviation (9%). Nine percent had QT prolongation and 8% had manifest pre-excitation. Eighty-one (33%) patients had tachyarrhythmia, 42 (17%) had ventricular tachycardia (VT), 14 (6%) had atrial tachycardia, and 19 (8%) had re-entrant supraventricular tachycardia (SVT). Four patients had atrial flutter and one had arterial fibrillation (AF). Only 25 of these 81 patients had arrhythmias during their presentation. A majority (56/81) developed during the follow up.

Five patients underwent radio frequency (RF) ablation of re-entrant SVT. Two patients with monomorphic VT underwent successful RF ablation without recurrence. Eleven patients received implantable cardioverter defibrillators, two of whom received transplants subsequently. During the study period, 31 (12.8%) died and 13 (5.4%) underwent cardiac transplantation. The incidence of death or transplantation in infantile LVNC was 25%. The five-year survival rate free from death or transplantation for patients with systolic dysfunction was 60% versus 98%, for those with normal cardiac function. The five-year survival rates were 63, 64, 86, and 98% for dilated, mixed, hypertrophic, and normal dimension phenotypes, respectively. The EKG abnormalities and arrhythmias were also strongly associated with the outcomes. Of the 42 children with documented VT, 18 (43%) died or received a transplant, representing a four-fold increase in this group. Fifteen (6.2%) patients experienced sudden cardiac death, none with normal cardiac dimensions and function.

This large report on the natural history of children with LVNC highlights the mortality profiles and incidence of arrhythmias and sudden cardiac death. EKG abnormalities are prominent and have diagnostic and prognostic implications. Preceding cardiac dysfunction or ventricular arrhythmias are associated with an increased risk of sudden death. Children with LVNC need life-long monitoring. Those with normal cardiac dimensions and function, without preceding arrhythmias, are likely to have a low risk for sudden death. The role of oral anticoagulation has not been evaluated in this study.

Transcatheter Potts Shunt Creation in Patients with Severe Pulmonary Arterial Hypertension: Initial Clinical Experience

Esch JJ, Shah PB, Cockrill BA, Farber HW, Landzberg MJ, Mehra MR, et al.

J Heart Lung Transplant 2013;32:381-7

Severe pulmonary artery hypertension (PAH) carries very high mortality. Creation of an atrial septal defect (ASD) as a palliation has been reported, but it does not reduce the pressure overload of the right ventricle, and hence, has a limited role. Creation of an anastomosis between the left pulmonary artery (LPA) and the descending thoracic aorta (DA), referred to as a Potts shunt, may present an alternative and effective palliative strategy. In this study, patients with intractable symptoms, despite advanced therapies and at risk of sudden death, were considered for transcatheter creation of the Potts Shunt (TPS). The authors describe their initial experience in four patients.

Procedure description: A pre-procedural computed tomography (CT) scan was performed in all patients, to locate the site of the LPA with DA. Using the Brockenbrough needle, the DA and LPA were pierced with a back end of the 0.014" Stabilizer wire, which was then snared at the LPA from the venous end. Using a 3.5 mm balloon, the sheath was advanced into the LPA. A 7 × 22 mm atrium covered stent was positioned to span the LPA and DA walls and deployed, which was then anchored by the elastic recoil of the aortic and LPA walls.

Of the seven patients considered for TPS, four were catheterized. This was successfully performed in three patients. The fourth patient developed fatal intrathoracic bleeding. Patient 1 was a 35-year-old, with idiopathic suprasystemic PAH. A dilated LPA was seen compressing the left main coronary artery, but was considered non-flow limiting. The TPS was successful. Four months later, the patient presented with angina and the left main was stented. His saturations in the ascending and descending aorta were 92 and 86%, respectively. Patient 2 was a 40-year-old man, with severe PAH, secondary to graft versus host disease following stem cell transplantation for acute myelogenous leukemia. The TPS was successful. However, he died on post-procedure day five due to ventilator-associated pneumonia and multi-organ failure. Patient 3: Was a18-year-old woman with idiopathic PAH, with recurrent near syncope. The TPS was successful; she was discharged on day two, with significant improvement in her symptoms at four months of follow up. The three additional patients who were considered for TPS died suddenly, before the procedure could be done.

The Transcatheter Potts Shunt could effectively reduce the resistance faced by the right ventricle (RV). As right-to-left shunting is into the DA, the brain and myocardium are not exposed to desaturated blood and paradoxic emboli. Many aspects of TPS are not clear, especially the size and length of the stent, for a given patient. The durability of a covered stent in this position is also unclear. The procedure appears technically demanding with significant risk of mortality. However, considering the bleak outcome of patients with refractory severe PAH, new strategies for RV salvage, like TPS, warrant further cautious evaluation.

Arterial Switch for Transposition with Left Outflow Tract Obstruction: Outcomes and Risk Analysis

Kalfa DM, Lambert V, Baruteau AE, Stos B, Houyel L, Garcia E, et al.

Ann Thorac Surg 2013;95:2097-103

Complete transposition of great arteries (TGA) can have associated left ventricular outflow tract obstruction (LVOTO), which could be valvar, subvalvar or both. Presence of significant LVOTO has been considered to be a contraindication for an arterial switch operation (ASO). However, feasibility of ASO in a subset of these patients has been demonstrated, eliminating the need for prosthetic reconstruction of the right ventricular outflow. This study is a retrospective evaluation of those children with TGA/LVOTO who underwent ASO.

Fifty-five patients operated between 1986 and 2011, were studied. Obstruction was defined as a peak gradient exceeding 20 mmHg on the preoperative echocardiogram. Forty (73%) of the patients were boys. Median age at surgery was 30 days (0-16 months) and weight was 3.7 kg (2-5.4). Seventy-eight percent had associated Ventricular Septal Defects (VSDs). The median LVOT gradient was 40 mmHg (20-112 mmHg) and the median PV z-score was 0 (- 2.8 to + 4.3). LVOTO was at valvar level in 16%, at the subvalvular level in 28%, and both valvar and subvalvular levels in 56%. Of the 55 patients, LVOTO intervention was done in 23 patients. It included resection of the subvalvar tissue in 15, accessory mitral valvar tissue in five or aneurysm of membranous septum in three, division of abnormal mitral chordae in three, neoaortic valvotomy in three, and neoaortic leaflet debridement in two. The median cardiopulmonary bypass (CPB) time was 152 ± 59 minutes and aortic clamping time was 92 ± 32 minutes. Median follow up was 7.9 ± 6.5 years.

Median length of Intensive Care Unit (ICU stay) was six days (1-20 days). Early mortality was seen in 11% at a median of 32 days. Two deaths were related to interventions for complex multilevel LVOTO. The other four deaths were related to myocardial infarction in two, pulmonary hypertension crisis in one, and hemorrhage in one. Late death occurred in one patient, who died of sepsis after a redo operation for mitral regurgitation. The mean LVOT gradient at the last follow up was 3 ± 9 mmHg, in three patients, it exceeded 20 mmHg. Moderate aortic regurgitation was present in seven (13%) patients. Eleven (20%) underwent 16 reoperations, of which four were related to LVOTO.

Recurrence of LVOTO was more in patients with an initial LVOTO related to the AV valve (25% vs. 5%, P = 005). LVOT intervention at the time of ASO had a significantly increased risk of long-term aortic regurgitation (26% vs. 3%, P = 0.03). A preoperative PV z-score of less than - 1.7 was an independent predictor of LVOTO. Preoperative PV z-score of less than - 1.8 and LVOTO related to accessory mitral valve tissue were independent predictors of recurrent LVOTO. The preoperative LVOT peak gradient and the presence of VSD did not play a role in predicting the outcomes. The actuarial survival was 89% and the actuarial freedom from moderate-to-severe aortic regurgitation was 76%.

In this large series of patients with TGA with LVOTO, who underwent ASO, the risk of recurrent LVOTO was only 5%, which appears much less than that reported with Rastelli operation (29%), which was more commonly done in this subset of patients. The authors conclude that in ASO with LVOTO, if the PV z-score exceeds - 1.8 and valvar or subvalvar tissue is resectable, ASO should be considered, regardless of the severity of the LVOT peak gradient and the presence of a VSD. However, the development of neoaortic regurgitation (13%) is a concern.

Clinical Features of Adult Patients with Eisenmenger's Syndrome in Japan and Korea

Sakazaki H, Niwa K, Nakazawa M, Saji T, Nakanishi T, Takamuro M, et al.

Int J Cardiol 2013;167:205-9

Disease Targeted Therapy (DTT) for pulmonary hypertension, including prostanoids, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors has been shown to improve functional capacity, oxygen saturation, and pulmonary hemodynamics. This multicenter study is aimed to observe the impact of DTT on the mortality and morbidity in patients with the Eisenmenger syndrome (ES).

Patients ≥ 16 years of age, who presented to the 16 participating institutes in Japan and Korea, from 1998 to 2009, were included. Of the 198 patients studied, 64% were females. The median age at the last clinic visit was 34 years (16-69). Seventy percent of them had simple defects (Atrial Septal Defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA)) and the rest had complex defects (Atrioventricular septal defect (AVSD), univentricular physiology, transposition of great arteries (TGA), double outlet right ventricle (DORV), Aortopulmonary (AP) window, Truncus). Fifty-eight (29%) had Trisomy 21. The mean SpO2 on room air was 85 ± 8.6%.

One hundred and seventeen (59%) received heart failure medication; digoxin (36%), loop diuretics (37%), spironolactone (24%), angiotensin converting enzyme inhibitor or receptor blocker (19%), calcium antagonist (6%), and four patients received beta blocker and pimobendan. Ninety-five patients (48%) were treated with DTT. Six patients received IV epoprostenol therapy and five received a combination therapy of bosentan and sildenafil. Fifty-four out of 117 (61%) showed clinical improvement; improved breathlessness in 25, increased six-minute walk distance in 17, increased resting SO2 in six, and improvement in the World Health Organization functional class (WHO FC) in three. In two patients, bosentan caused aggravation of heart failure. Two other patients on bosentan had worsening of functional class and three others, where bosentan was added on to Beroprost, had worsening of hypoxemia, needing withdrawal of the drug.

Five, 52, 36, 7, and 15% of the patients were in functional classes I, II, III, and IV, respectively, at the last clinic visit. During a median follow up of eight years, 30 patients died. Fourteen died suddenly, three had documented ventricular fibrillation, and there was pulmonary hemorrhage in one patient. The remaining 16 patients died due to, congestive heart failure (10), hypoxemia (4), pulmonary hemorrhage (1), and multi-organ failure (1). As expected, there was significant worsening of functional class and SpO 2 , with significant increase in the mean hematocrit. The survival rate in patients taking DTT was not significantly different from those without (87% vs. 84%, P = 0.55). Also, the survival rate did not differ in patients with simple versus complex anatomies or with or without trisomy 21.

In this large retrospective study on patients with ES, it was seen that advanced therapy for pulmonary hypertension could reduce the symptoms in half of those who received them and it worsened heart failure in a small percentage. These therapies did not reduce the mortality.

Correspondence Address:
Bhava RJ Kannan
23, Swagath Residency, Last East Cross Street, Anna Nagar, Madurai
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Source of Support: None, Conflict of Interest: None

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