Transcatheter closure of ruptured sinus of Valsalva aneurysm in a pregnant woman.

Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly that is usually congenital, but may be acquired. They are usually asymptomatic unless they compress adjacent structures, develop thrombosis, or rupture. A ruptured SVA (RSVA) can lead to rapid hemodynamic deterioration and often needs to be addressed emergently. Surgical correction has traditionally been the treatment of choice for RSVA; however, lately they have been successfully closed percutaneously using various transcatheter devices. Few cases of RSVA during pregnancy have been reported which were conservatively or surgically managed. There is no documented case of transcatheter closure of RSVA during pregnancy. We report the first case of successful percutaneous device closure of RSVA using an Amplatzer duct occluder in a pregnant woman presenting with heart failure due to RSVA at 26 weeks of gestation. .

Atrial baffle problems following the Mustard operation in children and young adults with dextro-transposition of the great arteries: the need for improved clinical detection in the current era.

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.

Postprocedural outcomes of rural children undergoing correction of congenital heart lesions in Yunnan Province, China.

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.

Electrocardiographic strain pattern in children with left ventricular hypertrophy: a marker of ventricular dysfunction.

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 (LVH(echo)) with ECG-strain pattern; group II (n = 54) comprised children with LVH(echo) 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 (> or = 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 LVH(echo). 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.

Effect of aerosolized PGE(1) on the ductus arteriosus of neonatal swine.

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) (1200ng/kg/min) [Study] or nebulized saline [Control] was administered using a jet nebulizer. Transthoracic echocardiography (TTE) was performed prior to (T0) and after 24h of aerosol therapy (T24). The DA was also evaluated histomorphologically at autopsy. RESULTS: Fifteen piglets, 1-9 days old (study=9; control=6), were evaluated for DA patency. Study piglets received IPGE(1) for 12-24h. TTE was performed on 12 piglets at T0. Nine animals showed no ductal flow 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 12-24h 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.

Longitudinal changes in heart rate-corrected measures of exercise performance in children.

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 (O(2) pulse), O(2) pulse at ventilatory anaerobic threshold, O(2) pulse at a heart rate of 140 beats per minute, and slope of the VO(2)-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 O(2 )pulse and slope of the VO(2)-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.

Sildenafil use in a symptomatic neonate with severe Ebstein's anomaly of the tricuspid valve.

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.

Prenatal diagnosis of complete atrioventricular septal defect with truncus arteriosus.

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.

Effectiveness of transvenous pacemaker leads placed through intravascular stents in patients with congenital heart disease.

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.

Abnormal origin of the right subclavian artery from the right pulmonary artery in a patient with D-transposition of the great vessels and left juxtaposition of the right atrial appendage: an unusual anatomical variant.

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.

Four-dimensional ultrasonography of the fetal heart using color Doppler spatiotemporal image correlation.

OBJECTIVE: To describe clinical and research applications of 4-dimensional imaging of the fetal heart using color Doppler spatiotemporal image correlation. METHODS: Forty-four volume data sets were acquired by color Doppler spatiotemporal image correlation. Seven subjects were examined: 4 fetuses without abnormalities, 1 fetus with ventriculomegaly and a hypoplastic cerebellum but normal cardiac anatomy, and 2 fetuses with cardiac anomalies detected by fetal echocardiography (1 case of a ventricular septal defect associated with trisomy 21 and 1 case of a double-inlet right ventricle with a 46,XX karyotype). The median gestational age at the time of examination was 21 3/7 weeks (range, 19 5/7-34 0/7 weeks). Volume data sets were reviewed offline by multiplanar display and volume-rendering methods. Representative images and online video clips illustrating the diagnostic potential of this technology are presented. RESULTS: Color Doppler spatiotemporal image correlation allowed multiplanar visualization of ventricular septal defects, multiplanar display and volume rendering of tricuspid regurgitation, volume rendering of the outflow tracts by color and power Doppler ultrasonography (both in a normal case and in a case of a double-inlet right ventricle with a double-outlet right ventricle), and visualization of venous streams at the level of the foramen ovale. CONCLUSIONS: Color Doppler spatiotemporal image correlation has the potential to simplify visualization of the outflow tracts and improve the evaluation of the location and extent of ventricular septal defects. Other applications include 3-dimensional evaluation of regurgitation jets and venous streams at the level of the foramen ovale.

Use of balloon pull-through technique to assist in CardioSEAL device closure of patent foramen ovale.

CardioSEAL device closure of patent foramen ovale (PFO) has been advocated for the treatment of patients with cryptogenic stroke. Using the standard delivery technique, partial deployment of the CardioSEAL device can occur, especially in patients with a thick septum secundum and/or long PFO tunnel. We hypothesized that using a left atrial-to-right atrial balloon pull-through to make the septum primum incompetent would result in improved final device position regardless of septal thickness or tunnel length. Catheterization reports, cineangiograms, and transesophageal echocardiograms of 51 patients who underwent CardioSEAL device closure of PFO between March 2000 and August 2002 were retrospectively reviewed. Group 1 (n = 21) included patients with CardioSEAL placement using the standard technique and group 2 (n = 30) included patients with CardioSEAL placement using the balloon pull-through technique. There were no differences between the groups in terms of age (43.6 vs. 45.3 years; P = NS), weight (83.3 vs. 89.9 kg; P = NS), septum secundum thickness (6.4 vs. 7.0 mm; P = NS), PFO tunnel length (15.5 vs. 13.1 mm; P = NS), or device size. In group 1, 4/21 (19%) had partial deployment of the CardioSEAL device, while in group 2, no partial CardioSEAL deployment (0/30) was observed. No complications were associated with the balloon pull-through technique. We conclude that the left atrial-to-right atrial balloon pull-through technique is safe and may allow for better final position of the CardioSEAL device during PFO closure.