Jennifer Co-Vu

Ventricular Septal Defect

BackgroundVentricular septal defects are the commonest congenital cardiac malformations. They can exist in isolation, but are also found as integral components of other cardiac anomalies, such as tetralogy of Fallot, double outlet right ventricle, or common arterial trunk. As yet, there is no agreement on how best to classify such defects, nor even on the curved surface that is taken to represent the defect.MethodsBased on our previous pathological and clinical experiences, we have reviewed the history of classification of holes between the ventricles. We proposed that the defects are best defined as representing the area of deficient ventricular septation. This then permits the recognition of clinically significant variants according to the anatomic borders, and the way the curved surface representing the area of deficient septation opens into the morphologically right ventricle.ResultsClinical manifestation depends on the size of the defect, and on the relationship between systemic and pulmonary vascular resistances. Symptoms include failure to thrive, along with the manifestations of the increase in flow of blood to the lungs. Diagnosis can be made by physical examination, but is confirmed by echocardiographic interrogation, which delineates the precise anatomy, and also provides the physiologic information required for optimal clinical decision-making. Cardiac catheterization offers additional information regarding hemodynamics, particularly if there is a concern regarding an increase in pulmonary vascular resistance. Hemodynamic assessment is rarely necessary to make decisions regarding management, although it can be helpful if assessing symptomatic adults with hemodynamically restrictive defects. In infants with defects producing large shunts, surgical closure is now recommended in most instances as soon as symptoms manifest. Only in rare cases is palliative banding of the pulmonary trunk now recommended. Closure with devices inserted on catheters is now the preferred approach for many patients with muscular defects, often using a hybrid procedure. Therapeutic closure should now be anticipated with virtually zero mortality, and with excellent anticipated long-term survival.ConclusionVentricular septal defects are best defined as representing the borders of the area of deficient ventricular septation. An approach on this basis permits recognition of the clinically significant phenotypic variants.

Long-term outcomes of the neoaorta after arterial switch operation for transposition of the great arteries.

BACKGROUND After the arterial switch operation (ASO) for transposition of the great arteries (TGA), the native pulmonary root and valve function in the systemic position, and the long-term risk for neoaortic root dilation and valve regurgitation is currently undefined. The aim of this study was to determine the prevalence and progression of neoaortic root dilation and neoaortic valve regurgitation in patients with TGA repaired with the ASO. METHODS Measurements of the neoaortic annulus, neoaortic root at the level of the sinuses of Valsalva, and the degree of neoaortic regurgitation were assessed by serial transthoracic echocardiograms on 124 patients with TGA at a median follow-up of 7.2 years (range, 1 to 23 years) after the ASO at our institution. RESULTS Neoaortic root dilation with z scores of 2.5 or greater was identified in 66%, and the root diameter z score increased at an average rate of 0.08 per year over time after ASO. Freedom from neoaortic root dilation at 1, 5, 10, and 15 years after ASO was 84%, 67%, 47%, and 32%, respectively. Risk factors for root dilation include history of double-outlet right ventricle (p = 0.003), previous pulmonary artery banding (p = 0.01), and length of follow-up (p = 0.04). Neoaortic valve regurgitation of at least moderate degree was present in 14%. Neoaortic root dilation was a significant risk factor for neoaortic valve regurgitation (p < 0.0001). No patient required reintervention on the neoaorta or neoaortic valve during follow-up. CONCLUSIONS Progressive neoaortic root dilation is common in patients with TGA after the ASO. Continued surveillance of this population is required.

A Rare Case of Hypoplastic Left Heart Syndrome and Scimitar Syndrome: Value of Neonatal Cardiac Magnetic Resonance Imaging

A 3-day-old neonate born at 37 weeks gestation was referred to our clinic for evaluation of a heart murmur. She was noted to have tachypnea with feeds but had no diaphoresis or cyanosis. At presentation, she was nondysmorphic and had minimal distress, with heart rate of 166 beats/min, a variable respiratory rate of 24–87 breaths/min, a blood pressure 60/30, and an oxygen saturation of 95% in room air. She weighed 2.4 kg (24th percentile). Her cardiovascular examination was significant for a right ventricular tap, with a grade 3/6 systolic ejection murmur best heard at the left midsternal border. There were no gallops or rubs. No organomegaly was noted. The patient’s chest X-ray showed cardiomegaly and an elevated right hemidiaphragm. The 12-lead electrocardiogram (ECG) showed sinus rhythm with right atrial enlargement, left axis deviation, and a paucity of left-sided forces. A transthoracic echocardiogram (TTE) showed hypoplastic left heart syndrome with aortic atresia and mitral atresia. The right pulmonary artery appeared to be hypoplastic. The left-sided pulmonary veins appeared to drain to the left atrium, and at least one right-sided pulmonary vein was seen draining to the inferior vena cava. The TTE and chest X-ray findings raised suspicion for scimitar syndrome associated with hypoplastic left heart syndrome (HLHS). The baby was admitted to the neonatal intensive care unit and started on prostaglandins. Cardiac magnetic resonance (CMR) imaging with magnetic resonance angiography (MRA) (Siemens Magnetom Symphony magnet, Munich, Germany) was performed with the patient under general anesthesia to define further the right lung arterial supply and the pulmonary venous drainage. The CMR confirmed the diagnosis of HLHS with mitral and aortic atresia as well as the rare finding of associated scimitar syndrome. The CMR also showed dextroposition of the heart with hypoplastic right lung (50% of normal size), anomalous drainage of the right pulmonary veins to the inferior vena cava (Fig. 1), a small right pulmonary artery, and a single aortopulmonary (AP) collateral (thought to be similar in size to the celiac artery) supplying the right lower lung from the descending abdominal aorta (Fig. 2). Functional information, provided by CMR, showed the right ventricular ejection fraction to be low normal (47%),

Maternal hyperoxygenation: A potential therapy for congenital heart disease in the fetuses? A systematic review of the current literature

To assess efficacy, safety, outcomes, and intrauterine complications following maternal hyperoxygenation (MH) therapy in fetuses with congenital heart disease (CHD).

Effect of prenatal diagnosis on hospital costs in complete transposition of the great arteries

Prenatal diagnosis of congenital heart disease (CHD) is associated with improved clinical outcomes, yet its impact on the cost of hospitalization is not well described. We hypothesized that prenatal diagnosis of complete transposition of the great arteries (d‐TGA) results in lower total hospital costs compared with postnatal diagnosis.

Left Ventricular Aneurysm Following Blunt-Force Trauma in a Child

Graphical abstract

Left Ventricular Metastasis in Neuroblastoma: A Case Report.

Neuroblastoma is the most common extracranial solid tumor in children. Most common sites of metastases from neuroblastoma are bone marrow, bone and lymph nodes, however cardiac metastasis is rarely seen. Metastatic cardiac tumors are 20 to 40 times more common than primary cardiac tumors. Mechanism of cardiac metastasis can be hematogenous, lymphatogenous, and direct extension/infiltration of tumor cells. Usually right heart metastasis is seen. Left ventricular metastatic tumor has never been reported with neuroblastoma.

Generating patient-specific pulmonary vascular models for surgical planning

Each year in the U.S., 7.4 million surgical procedures involving the major vessels are performed. Many of our patients require multiple surgeries, and many of the procedures include “surgical exploration”. Procedures of this kind come with a significant amount of risk, carrying up to a 17.4% predicted mortality rate. This is especially concerning for our target population of pediatric patients with congenital abnormalities of the heart and major pulmonary vessels. This paper offers a novel approach to surgical planning which includes studying virtual and physical models of pulmonary vasculature of an individual patient before operation obtained from conventional 3D X-ray computed tomography (CT) scans of the chest. These models would provide clinicians with a non-invasive, intricately detailed representation of patient anatomy, and could reduce the need for invasive planning procedures such as exploratory surgery. Researchers involved in the AirPROM project have already demonstrated the utility of virtual and physical models in treatment planning of the airways of the chest. Clinicians have acknowledged the potential benefit from such a technology. A method for creating patient-derived physical models is demonstrated on pulmonary vasculature extracted from a CT scan with contrast of an adult human. Using a modified version of the NIH ImageJ program, a series of image processing functions are used to extract and mathematically reconstruct the vasculature tree structures of interest. An auto-generated STL file is sent to a 3D printer to create a physical model of the major pulmonary vasculature generated from 3D CT scans of patients.

Cardiac magnetic resonance imaging can detect early vascular changes in children with type 1 diabetes (T1DM)

Adult studies have shown that cardiac magnetic resonance (CMR) can image vascular changes including altered aortic compliance and early plaque which correlates well with Framingham risk score and endothelial function. Impaired endothelial function, measured with brachial artery reactivity testing, is recognized as an early and modulating process in the pathophysiology of atherosclerotic plaque development. No CMR imaging studies of early atherosclerosis and vascular health exist in diabetic pediatric populations. This prospective pilot CMR proof of concept study hypothesized that children with T1DM will have thoracic aortic wall characteristics, as well as CMR-derived aortic computational fluid dynamic (CFD) models, different from age-matched control subjects. Furthermore, a positive correlation was sought between key CMR data and brachial artery reactivity measures, as well as cardiac venous biomarkers in T1DM. 7 control and 8 T1DM pediatric subjects had same-day fasting CMR scan, brachial artery testing, and venous blood draw (for lipid panel, HgbA1c, glucose, high sensitivity c-reactive protein (hs-cRP), fibrinogen, and homocysteine). Enrolled T1DM and control subjects were similar: age (14.5 ±1.7 versus (vs.) 15.2 ± 2.7 years), sex (4 male and 4 female T1DM vs. 5 male and 2 female controls), weight (55.7 ± 14.8 vs. 67.6 ± 23.5 kg), body mass index (20.8 ± 4.3 vs. 23.1 ± 5.7 kg/m2) and systolic blood pressure (113 ± 11.4 vs. 116 ± 8.8). They had no significant differences in lipid values, fibrinogen, or hs-cRP, but did differ with regard to glucose (182.1 ± 100.8 vs. 85.4 ± 5.7, p < 0.02), HgbA1c (9.0 ± 2.2 vs. 5.2 ± 0.3, p < 0.001), and homocysteine (4.3 ± 0.6 vs. 5.7 ± 1.0, p < 0.04). While brachial artery reactivity did not change with age, CMR determined ascending aortic (AAo) compliance decreased with increasing age in T1DM (R2 = 0.54, p < 0.09), but not for controls. In T1DM, CMR determined ascending aortic (AAo) compliance declined, as hs-cRP increased (R2 = 0.66, p < 0.05).No other significant correlations existed between AAo compliance and venous biomarkers. Preliminary qualitative analyses of aortic CFD models show different patterns of aortic wall shear stress and oscillatory shear index (OSI) for T1DM versus age-matched control pediatric subjects (Figure ​(Figure1).1). In conclusion, this pilot CMR study of T1DM and control pediatric subjects illustrates that early differences in vascular characteristics can be detected by MRI and correlate with age and with select venous biomarkers. Figure 1