The management of congenital heart disease (CHD) remains a significant challenge in developing regions. Since 2006, China California Heart Watch has provided cardiac services in China’s Yunnan province. Our Grants for Kids program aims to diagnose and fund surgical and nonsurgical treatments for underprivileged children with congenitally malformed hearts. This report analyzes our patient outcomes. From 2007 to 2010, 36 children with CHD underwent either surgical or percutaneous procedures at local Chinese medical centers, and 94% of our patients could be contacted for follow-up assessment. The mortality and complication rates of our patient population compare favorably with international data. Our study provides a model through which networking with local hospitals and regional cardiac centers can be an effective way to assist developing areas in providing cardiac care to rural underserved populations.

OBJECTIVE Intraatrial Mustard baffle repair of dextro-transposition of the great arteries (d-TGA) is vulnerable to complications, typically obstruction and leaks. Because patients often require pacemakers or intracardiac electrophysiology studies (EPS)/ablation for arrhythmias, narrowed or obstructed baffles restrict cardiac access hindering intracardiac procedures. Current guidelines recommend clinical as well as comprehensive transthoracic echocardiographic/Doppler (TTE) studies to identify baffle problems. This study reviews the effectiveness of these guidelines in detection of baffle issues pre-EPS catheterization and need for ancillary vascular interventions. DESIGN Data from all patients with repaired d-TGA referred for hemodynamic catheterization or EPS between 1995 and 2009 at our institution were reviewed, including symptoms and TTE findings. Obstruction was defined as either a disturbed color Doppler flow or mean velocity >1 m/s above the mitral valve or directly measured pressure gradient >4 mm Hg or more than 50% baffle diameter narrowing by venography. RESULTS Of 59 patients (34 pacemaker, 9 ablation, 16 routine hemodynamic) ages 8-39 years (mean 22.8), only three (5%) had symptoms of obstruction. However, baffle complications were found in 33 patients (56%), some with more than one problem: superior vena cava (SVC) obstruction in 32, inferior VC in two and leak in four. Baffle stenting was required in 24 patients and leak closure in two. Precatheterization TTE was available in 51 patients and showed 34% sensitivity, 61% specificity, 63% negative predictive value, and only 37% positive predictive value in recognizing baffle complications when compared with the actual catheterization findings. CONCLUSION This study reports that baffle complications in patients with d-TGA following Mustard operation are more common than previously reported. However, comprehensive TTE and clinical symptoms are not effective enough to recognize these complications. Suspicion of and better noninvasive imaging prior to catheterization is required.

The objective of this study was to assess the relation between strain pattern on electrocardiogram (ECG-strain) and echocardiographic indices of left ventricular (LV) structure and function in children with LV hypertrophy (LVH). ECG-strain is a marker of LVH and is associated with adverse cardiovascular prognosis in adults. The significance of ECG-strain and its relation to LV structure and function has not been studied in children. We retrospectively analyzed electrocardiograms (ECGs) and echocardiograms of 101 children enrolled in this study. Subjects were divided into three groups: group I (n = 21) comprised children with LVH confirmed by echocardiography (LVHecho) with ECG-strain pattern; group II (n = 54) comprised children with LVHecho without ECG-strain pattern; and group III (n = 26) comprised children without LVH (control group). ECG-strain was defined as a down-sloping convex ST-segment depression (≥0.1 mV) with an inverted asymmetrical T-wave opposite to the QRS axis in leads V5 and/or V6. LV structure and function was measured using conventional and tissue Doppler echocardiography. ECG-strain was associated with greater interventricular septal thickness, posterior wall thickness, and LV mass index (LVMI) compared with those without ECG-strain (P < 0.0001 for each variable). Concentric LVH was more common in those with ECG-strain (16 of 21 vs. 9 of 54 patients; P = < 0.0001). ECG-strain was associated with systolic, diastolic, and combined systolic–diastolic dysfunction in children with LVHecho. Among children with LVH, ECG-strain is associated with higher LVMI, concentric pattern of LVH, and LV systolic and diastolic dysfunction. Whether this has similar adverse prognostic implications as it does in adults remains to be determined.

Fetal arrhythmias are diagnosed in 1 to 3% of pregnancies, of which supraventricular tachycardia (SVT) constitutes 10% (Reed, 1989). Sustained fetal SVT with a heart rate more than 220 beats per minute (bpm) can result in congestive heart failure and nonimmune hydrops, which is associated with a poor outcome (Simpson and Sharland, 1998). SVT in twin pregnancy is infrequently reported and poses a therapeutic and ethical dilemma in regard to the effect of treatment on the unaffected twin. To date, there is no evidence that fetal SVT in one twin affects hemodynamics of the other twin who is in normal rhythm. We report for the first time, a case in which fetal SVT in one twin was associated with early signs of congestive heart failure, in the other twin, with subsequent echocardiographic improvement following control of the SVT in the first twin. A 26-year-old gravida 2, para 0 African-American woman with a twin pregnancy at 27 (3/7) weeks of gestation was referred to us for evaluation of fetal tachycardia in one of the twins. The course of pregnancy was uneventful until then. Specifically, there was no history of hypertension, diabetes or infections. She was a nonsmoker and denied use of alcohol or drugs. She was not on any medications. During a regular prenatal visit, auscultation revealed audible arrhythmia in twin A with heart rate in the range of 200 bpm. Ultrasound revealed monochorionic diamniotic twins of similar size (estimated fetal weight of twin A, 992 g and twin B, 959 g). There were no other congenital defects. A fetal echocardiogram utilizing M mode and Doppler techniques revealed that Twin A had SVT at a rate of 230 bpm with 1 : 1 conduction to ventricles. Cardiac anatomy was normal. The size and contractile function of the ventricles were normal and there was no mitral or tricuspid regurgitation. There was no evidence of pericardial effusion or congestive heart failure as assessed by the cardiovascular profile (Huhta, 2004). Fetal echocardiography of Twin B revealed normal cardiac structural anatomy. There was mild cardiomegaly with a cardiothoracic circumference ratio of

Background: Inhaled PGE(1) (IPGE(1)) is a potential pulmonary vasodilator in neonatal respiratory failure. However, its effect on the patency of the ductus arteriosus (DA) has not been described.Objective: To investigate the effect of IPGE(1) on the DA in healthy piglets.Design/methods: IPGE(1) (1200 ng/kg/min) [Study] or nebulized saline [Control] was administered using a jet nebulizer. Transthoracic echocardiography (TTE) was performed prior to (TO) and after 24 h of aerosol therapy (T24). The DA was also evaluated histomorphologicaily at autopsy.Results: Fifteen piglets, 1-9 days old (study = 9: control = 6), were evaluated for DA patency. Study piglets received IPCE1 for 12-24 h. TTE was performed on 12 piglets at T0. Nine animals showed no ductal now and 3 (1 study, 2 control) had a small DA. TTE at T24 in 5 animals showed no change in DA. At autopsy, the ductal diameter and histologic maturity stage were comparable in study and control animals.Conclusions: High dose IPGE(1) given for 12-24 h does not exert significant effect on the DA of healthy term piglets as evaluated by echocardiography and histomorphology. We conclude that ductal patency in neonates is influenced not only by prostaglandins but also by factors like hypoxemia, prematurity, and heart disease. (C) 2009 Elsevier Inc. All rights reserved

OBJECTIVE:Pulmonary venous Doppler (PVD) patterns are abnormal in fetuses with hypoplastic left heart syndrome (HLHS) with restricted foramen ovale (rFO) when compared with healthy fetuses. The objective of this study was to define PVD patterns in HLHS fetuses with an unrestricted or patent foramen ovale (pFO).DESIGN:27 fetuses with HLHS and 66 healthy fetuses underwent echocardiography between 19 and 38 weeks of gestation. The pulmonary venous peak systolic (S), diastolic (D) and atrial reversal (A) velocities were measured; S/D ratio, velocity time integral of forward (VTI(f)) and reversed (VTI(r)) flows and VTI(r) expressed as percentage of VTI(f) (%R) were calculated. Independent examiners classified HLHS subjects into HLHS-pFO and HLHS-rFO (rFO or intact atrial septum).SETTING:Tertiary referral centre for paediatric cardiology.RESULTS:Compared with healthy controls, the HLHS-pFO group (n = 16) had higher S (32.9 (3.2) vs 23.5 (1.6) cm/s (adjusted mean (SE)); p = 0.01), A (10.5 (3.4) vs 0.17 (1.6) cm/s; p = 0.01), VTI(r) (0.78 (0.3) vs 0.01 (0.13) cm; p = 0.01) and %R (14.2% (3.2 %) vs -1.3% (1.5%); p<0.001). D velocity, S/D and VTI(f) showed no difference. In HLHS-rFO, further increase in S, A, VTIr and %R, decrease in D, increase in S/D and no change in VTI(f) were noted.CONCLUSIONS:PVD flow patterns are abnormal in HLHS even in the absence of rFO, suggesting that factors other than impaired left atrial egress play a role. Future studies of PVD patterns can provide important insights into left atrial dynamics, pulmonary venous return and pulmonary vascular development in fetal HLHS.

Ebstein's anomaly (EA) is associated with poor outcome in symptomatic neonates. Management typically includes prostaglandins or surgical creation of a Blalock-Taussig shunt. Right ventricular afterload reduction may help by improving the forward flow across the pulmonary valve. We report the successful use of oral sildenafil, a pulmonary vasodilator, in a neonate with severe EA.

Interpretation of change in exercise performance over time in children with repaired congenital heart disease is often hampered by poor effort that limits the maximum heart rate; this is often difficult to distinguish from chronotropic impairment, a common finding in these children. In an attempt to address this limitation, we sought to examine measures of exercise performance that are corrected for heart rate in healthy children and to determine if these change with somatic growth. We studied two serial graded exercise tests in 24 healthy children at an interval of >3 years. Paired comparisons revealed that maximum oxygen pulse (O2 pulse), O2 pulse at ventilatory anaerobic threshold, O2 pulse at a heart rate of 140 beats per minute, and slope of the VO2–heart rate relationship all increased with age. However, when indexed to somatic growth, there was no change in the mean values of these parameters over time. We conclude that O2 pulse and slope of the VO2–heart rate relationship during exercise increase in proportion to somatic growth in children so that optimal oxygen delivery to the exercising muscles is ensured. This study provides the “normative” response of exercise parameters to growth, against which responses of children with repaired congenital heart disease may be compared.

Truncus arteriosus (TA) is a rare cardiac anomaly constituting less than 1% of all congenital heart defects. Its association with complete atrioventricular septal defect (AVSD) is extremely unusual and only 12 cases diagnosed postnatally or postmortem have been reported so far. We describe the first case of truncus arteriosus with AVSD to be diagnosed prenatally by fetal echocardiography. Copyright © 2007 John Wiley & Sons, Ltd.

We describe a neonate with ductal-dependent congenital heart disease on extracorporeal membrane oxygenation (ECMO) for persistent pulmonary hypertension, who required markedly high doses of prostaglandin E 1 (PGE 1 ) to maintain patency of the ductus arteriosus: The effects of ECMO on the pharmacokinetics of PGE 1 are discussed.

Although effective to correct newborn cyanosis, the intra-atrial baffle or Mustard surgery for D-transposition of the great arteries (DTGA) is often associated with symptomatic rhythm disturbances requiring intracardiac (IC) ablation (AB) or pacemaker (PM) therapy for sinus or AV node dysfunction among patients (pts) surviving to young adulthood. However, AB or PM implant may be complicated by associated but undetected and asymptomatic atrial baffle problems. In such situations, the azygous vein often serves to decompress any superior or inferior vena cava (VC) baffle obstruction (obsr). Between 1995-2005, 41 pts, age 12-43y (median 22) were referred for IC study for AB or PM implant. Pre IC evaluations included transthoracic echocardiogram (ECHO) and Doppler (D) indices of VC-atrial baffle blood flow. All pts underwent IC hemodynamic and angiographic studies prior to AB (n=12) and/or PM (n=37) implant. IC studies revealed baffle narrowing (2-10mm, mean 6) compared with VC diameters (12-20mm, mean 15) (p<.05) and obsr (VC-atrial pressure gradient 5 -12 mmHg, mean 6) in 13/41 pts (32%) and a baffle leak (Qp/Qs 1.5:1) in 1/41(2%) requiring intervention. Pre-IC study ECHO/D in these pts identified only mild baffle flow changes in 8 and normal flow in 5 pts. All Obsr were treated during the same IC study with intravascular stenting to relieve the pressure gradients (mean 6 before vs. 0.7mmHg after, p <.05). The baffle leak was closed with an Amplatzer™ device. AB or PM implant were then performed without complications immediately after. This study stresses the need to recognize that non-invasive evaluations of young adults with DTGA and Mustard repair may be ineffective to detect significant atrial baffle problems. Because of location, VC and baffle studies may not be optimal by ECHO and azygous flow can minimize obsr baffle D flow patterns. IC studies for AB or PM implant in pts with DTGA/ Mustard repair should be performed with anticipation of need for concomitant intracardiac devices and done in institutions with interventional catheterization expertise equipped to treat these issues in adults with repaired congenital heart.

Eight patients with venous obstruction secondary to Mustard baffle obstruction or previous transvenous pacemaker leads underwent intravascular stent relief of their obstructions followed by the insertion of new leads. Patients were followed from 1.3 to 6.3 years (median 3) by clinical, hemodynamic, angiographic, and intravascular ultrasound methods and pacemaker evaluations. The median stent patency was 84%, with 1 patient developing complete stent occlusion. Pacing energy thresholds and impedances remained unchanged.

Clinical CardiologyVolume 27, Issue 7 p. 411-411 Images in CardiologyFree Access Pericardial yolk sac tumor presenting as cardiac tamponade in a 21-month-old child Richard A. Kerensky M.D., Richard A. Kerensky M.D. Divisions of Cardiology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this authorKavitha Chintala M.D., Kavitha Chintala M.D. Divisions of Cardiology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this authorDavid A. Bloom M.D., David A. Bloom M.D. Divisions of Radiology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this authorHenry L. Walters III M.D., Henry L. Walters III M.D. Divisions of Cardiovascular Surgery, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this authorMichael D. Pettersen M.D., Michael D. Pettersen M.D. Divisions of Cardiology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this author Richard A. Kerensky M.D., Richard A. Kerensky M.D. Divisions of Cardiology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this authorKavitha Chintala M.D., Kavitha Chintala M.D. Divisions of Cardiology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this authorDavid A. Bloom M.D., David A. Bloom M.D. Divisions of Radiology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this authorHenry L. Walters III M.D., Henry L. Walters III M.D. Divisions of Cardiovascular Surgery, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this authorMichael D. Pettersen M.D., Michael D. Pettersen M.D. Divisions of Cardiology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan, USASearch for more papers by this author First published: 05 December 2006 https://doi.org/10.1002/clc.4960270709Citations: 4AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume27, Issue7July 2004Pages 411-411 RelatedInformation

Isolation of the left subclavian artery (LSCA) or its anomalous origin from the pulmonary artery (PA) has been documented in several cases, especially in association with a right-sided aortic arch. Similar anomalies involving the right subclavian artery (RSCA) are less frequent. Anomalous origin of the RSCA from the PA in association with D-transposition of the great arteries (D-TGA) is exceedingly rare and only two cases have been reported so far. We present here, a case of aberrant origin of the RSCA from the right PA in a patient with D-TGA, in whom the diagnosis was rendered difficult due to the partial occlusion of the intervening ductus arteriosus (DA). We discuss the embryological basis of this anomaly and review its clinical and surgical implications.