Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension.

BACKGROUND: The placement of a pulmonary-to-systemic arterial shunt in children with severe pulmonary hypertension (PH) has been demonstrated, in relatively small studies, to be an effective palliation for their disease. OBJECTIVES: The aim of this study was to expand upon these earlier findings using an international registry for children with PH who have undergone a shunt procedure. METHODS: Retrospective data were obtained from 110 children with PH who underwent a shunt procedure collected from 13 institutions in Europe and the United States. RESULTS: Seventeen children died in-hospital postprocedure (15%). Of the 93 children successfully discharged home, 18 subsequently died or underwent lung transplantation (20%); the mean follow-up was 3.1 years (range: 25 days to 17 years). The overall 1- and 5-year freedom from death or transplant rates were 77% and 58%, respectively, and 92% and 68% for those discharged home, respectively. Children discharged home had significantly improved World Health Organization functional class (P < 0.001), 6-minute walk distances (P = 0.047) and lower brain natriuretic peptide levels (P < 0.001). Postprocedure, 59% of children were weaned completely from their prostacyclin infusion (P < 0.001). Preprocedural risk factors for dying in-hospital postprocedure included intensive care unit admission (hazard ratio [HR]: 3.2; P = 0.02), mechanical ventilation (HR: 8.3; P < 0.001) and extracorporeal membrane oxygenation (HR: 10.7; P < 0.001). CONCLUSIONS: A pulmonary-to-systemic arterial shunt can provide a child with severe PH significant clinical improvement that is both durable and potentially free from continuous prostacyclin infusion. Five-year survival is comparable to children undergoing lung transplantation for PH. Children with severely decompensated disease requiring aggressive intensive care are not good candidates for the shunt procedure.

Interruption of the Ascending Aorta-Biventricular Surgical Repair.

Interruption of the ascending aorta is an extremely rare anomaly defined by a point of interruption between the intrapericardial and extrapericardial aorta and can be explained by developmental errors proximal to the embryologic right aortic sac. Herein, we present a case of interruption of the ascending aorta and describe a successful biventricular surgical repair of this unique anomaly.

Increased Hepatic Stiffness in Young Adults After Biventricular Repair of Congenital Heart Disease.

BACKGROUND: This study evaluated hepatic stiffness by shear wave elastography to investigate subclinical hepatic changes in a cohort of patients with congenital biventricular heart disease (BHD). METHODS: The BHD patients and age-matched healthy controls were prospectively recruited for hepatic ultrasonography and shear wave elastography. Real-time B-mode imaging with Doppler was performed for celiac axis, superior mesenteric artery, and main portal vein, and hepatic shear wave elastography was assessed. Vascular Doppler indices included peak velocities; velocity time integral, resistive, pulsatility, and acceleration indices; and portal vein volumetric flow. One-way analysis of variance was used for comparisons between controls, BHD, and a cohort of Glenn and Fontan patients. RESULTS: In all, 66 subjects were included. Thirty-six subjects were in the BHD group (male, 25; female, 11; mean age 27.4 ± 4.6 years; mean weight 76.8 ± 18.5 kg), and 30 were healthy controls (male, 11; female, 23, mean age 27.4 ± 3.8 years; mean weight 70 ± 17.2 kg). Shear wave elastography was increased in BHD (8.11 ± 2.07 kPa) compared with controls (5.44 ± 1.18 kPa; P < .001). Hepatic stiffness in BHD was significantly different from that in the Fontan cohort but not in the infant Glenn cohort. CONCLUSIONS: Increased hepatic stiffness was observed in young adults with repaired BHD. Although cause is not established, possibilities include hepatic congestion early in life or elevated central venous pressures due to right heart burden. Further research is required to determine whether these patients will ultimately have clinically relevant liver disease.

Increased hepatic stiffness as consequence of high hepatic afterload in the Fontan circulation: a vascular Doppler and elastography study.

UNLABELLED: Hepatic dysfunction is a recognized complication after Fontan palliation of congenital heart disease. We sought to quantitatively measure hepatic stiffness and vascular Doppler indices using ultrasound (US) and shear wave elastography (SWE) in a Fontan cohort. Subjects were prospectively recruited for echocardiography and real-time hepatic duplex US with SWE for hepatic stiffness (kPa). Doppler peak velocities, velocity time integral, resistive, pulsatility, acceleration indices (RI, PI, AI), and flow volume were measured in celiac artery, superior mesenteric artery, and main portal vein (MPV). A subset underwent cardiac catheterizations with liver biopsy. Correlations were explored between SWE, duplex, hemodynamic, and histopathologic data. In all, 106 subjects were studied including 41 patients with Fontan physiology (age 13.8 ± 6 years, weight 45.4 ± 23 kg) and 65 controls (age 15.0 ± 8.4 years, weight 47.9 ± 22 kg). Patients with Fontan physiology had significantly higher hepatic stiffness (15.6 versus 5.5 kPa, P < 0.0001), higher celiac RI (0.78 versus 0.73, P = 0.04) superior mesenteric artery RI (0.89 versus 0.84, P = 0.005), and celiac PI (1.87 versus 1.6, P = 0.034); while MPV flow volume (287 versus 420 mL/min in controls, P = 0.007) and SMA AI (829 versus 1100, P = 0.002) were lower. Significant correlation was seen for stiffness with ventricular end-diastolic pressure (P = 0.001) and pulmonary artery wedge pressure (P = 0.009). Greater stiffness correlated with greater degrees of histopathologic fibrosis. No significant change was seen in stiffness or other duplex indices with age, gender, time since Fontan, or ventricular morphology. CONCLUSION: Elevated hepatic afterload in Fontan, manifested by high ventricular end-diastolic pressures and pulmonary arterial wedge pressures, is associated with remarkably increased hepatic stiffness, abnormal vascular flow patterns, and fibrotic histologic changes. The MPV is dilated and carries decreased flow volume, while the celiac and superior mesenteric arterial RI is increased. SWE is feasible in this population and shows promise as a means for predicting disease severity on liver biopsy.

Outcomes of congenital diaphragmatic hernia in the modern era of management.

OBJECTIVE: To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently. Degree of PH and survival were tested for association with clinical variables using Fischer exact test, χ(2), and regression analysis. RESULTS: Two hundred twenty patients met inclusion criteria. Worse PH measured at 1 month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation, patients inborn at the treating center, and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes. CONCLUSIONS: Severity of PH is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status, and need for extracorporeal membrane oxygenation.

Patent ductus arteriosus closure using the Amplatzer® vascular plug II for all anatomic variants.

OBJECTIVES: To evaluate the safety and efficacy of the Amplatzer® Vascular Plug II (AVPII) for closure of the patent ductus arteriosus (PDA). BACKGROUND: The PDA has significant anatomic variation. No device has proven applicable to all PDAs. Previous case reports and small series have documented limited use of the AVPII for some PDA types. We describe the largest and most diverse experience using the AVPII. METHODS: A retrospective analysis of patients undergoing percutaneous PDA closure between 01/01/2009 and 05/01/2012 was performed. The PDA was characterized, measured, and the device chosen was listed. Deployment technique, complications and procedural results were recorded. RESULTS: Sixty-seven procedures were performed. The AVPII was utilized for 43 (64.2%), 15 (20.9%) had coils, 7 (10.4%) had the AGA duct occluder, and 3 (4.5%) were referred for surgery. The AVPII was placed in infants as young as 2 months and 4.2 kg. AVPII size ranged from 4 to 10 mm. All PDA types were closed. Retrograde and antegrade deployments were performed, using the outer disc as a "retention skirt" to secure the device and improve occlusion. Three patients were up-sized prior to release. All deployments were successful; 89% "in-lab" and 100% closure on postprocedural echocardiogram. There were no complications. CONCLUSIONS: We report the largest experience with the AVPII for PDA closure. The device was used in all morphologic types and small patients. It is low profile, easily repositioned, and had excellent results without complications. We contend that this is the most versatile device currently available.

Reduced global longitudinal and radial strain with normal left ventricular ejection fraction late after effective repair of aortic coarctation: a CMR feature tracking study.

We sought to determine whether global and regional left ventricular (LV) strain parameters were altered in repaired coarctation of the aorta (COA) with normal LV ejection fraction (EF) when compared with healthy adult controls, and whether such alterations were related to LV hypertrophy (LVH). We identified 81 patients after COA repair (31 female, age 25 ± 8.5 years) with inclusion criteria at follow-up CMR of: age ≥13 years, time post-repair ≥10 years, no aortic valve disease, LV-EF >50%). LV deformation indices derived using CMR-feature tracking and volumetric EF were compared between COA patients and normal controls (n = 20, 10 female, age 37 ± 7 years), and between COA with versus without LVH. In repaired COA versus controls, LV-EF (%) was 62 ± 7.2 versus 58 ± 3.0 (p = 0.01), and LV mass (g/m(2)) 66 ± 16.8 versus 57.7 ± 6.0 (p = 0.0001). LV global longitudinal strain (GLS) was decreased to -17.0 ± 4.7% in COA (-20 ± 5% in controls, p = 0.02), and global radial strain (GRS) reduced to 40 ± 15% (50 ± 12.4% in controls, p = 0.003). The global circumferential strain (GCS) was preserved in COA at -23 ± 4.7% (-24.6 ± 2.4% in controls, p = 0.14). Regionally, LS decrease was marked in the basal segments (septal, p = 0.005, lateral, p = 0.013). In COA with LVH (n = 45, mass 76.3 ± 12.8 g/m(2)) versus without LVH (n = 36, mass 52.2 ± 10 g/m(2)), GLS was more markedly decreased (-15.7 ± 4.8 vs. -18.5 ± 4.2%, p = 0.016, but GRS and GCS were similar (p = 0.49 and 0.27). In post-repair COA with normal LV-EF, GLS and GRS are reduced whilst GCS is preserved. GLS reduction is more pronounced in the presence of LVH. GLS may qualify as indicator of early LV dysfunction.

Safety of cardiac magnetic resonance and contrast angiography for neonates and small infants: a 10-year single-institution experience.

BACKGROUND: With increasing applications of cardiac magnetic resonance (CMR) and magnetic resonance angiography (MRA) for evaluation of congenital heart disease (CHD), safety of this technology in the very young is of particular interest. OBJECTIVE: We report our 10-year experience with CMR in neonates and small infants with particular focus on the safety profile and incidence of adverse events (AEs). MATERIALS AND METHODS: We reviewed clinical, anesthesia and nursing records of all children ≤120 days of age who underwent CMR. We recorded variables including cardiac diagnosis, study duration, anesthesia type and agents, prostaglandin E1 (PGE1) dependence and gadolinium (Gd) use. Serially recorded temperature, systemic saturation (SpO(2)) and cardiac rhythm were analyzed. Primary outcome measure was any AE during or <24 h after the procedure, including minor AEs such as hypothermia (axillary temperature ≤95 °F), desaturation (SpO(2) drop ≥10% below baseline) and bradycardia (heart rate ≤100 bpm). Secondary outcome measure was unplanned overnight hospitalization of outpatients. RESULTS: Children (n = 143; 74 boys, 69 girls) had a median age of 6 days (1-117), and 98 were ≤30 days at the time of CMR. The median weight was 3.4 kg (1.4-6 kg) and body surface area 0.22 m(2) (0.13-0.32 m(2)). There were 118 (83%) inpatients (108 receiving intensive care) and 25 (17%) outpatients. Indications for CMR were assessment of aortic arch (n = 57), complex CHD (n = 41), pulmonary veins (n = 15), vascular ring (n = 8), intracardiac mass (n = 8), pulmonary artery (n = 7), ventricular volume (n = 4), and systemic veins (n = 3). CMR was performed using a 1.5-T scanner and a commercially available coil. CMR utilized general anesthesia (GA) in 86 children, deep sedation (DS) in 50 and comforting methods in seven. MRA was performed in 136 children. Fifty-nine children were PGE1-dependent and 39 had single-ventricle circulation. Among children on PGE1, 43 (73%) had GA and 10 (17%) had DS. Twelve children (9%) had adverse events (AEs)-one major and 11 minor. Of those 12, nine children had GA (10%) and three had DS (6%). The single major AE was respiratory arrest after DS in a neonate (resuscitated without sequelae). Minor AEs included desaturations (n = 2), hypothermia (n = 5), bradycardia (n = 2), and bradycardia with hypoxemia (n = 2). Incidence of minor AEs was 9% for inpatients (vs. 4% for outpatients), and 8% for neonates (vs. 9% for age ≥30 days). Incidence of minor AEs was similar between PGE1-dependent infants and the non-PGE1 group. There were no adverse events related to MRA. Of 25 outpatients, 5 (20%) were admitted for overnight observation due to desaturations. CONCLUSION: CMR and MRA can be accomplished safely in neonates and infants ≤120 days old for a wide range of pre-surgical cardiac indications. Adverse events were unrelated to patient age, complexity of heart disease, type of anesthesia or PGE1 dependence.

Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease.

AIMS: We sought to evaluate the efficacy of ultrasound contrast (UC) and low mechanical index real-time perfusion (RTP) in the haemodynamic and anatomic assessment of repaired congenital heart disease (CHD) at rest and during supine bicycle stress echocardiography (BSE). METHODS AND RESULTS: Patients with CHD (n = 51, median age 21.5 years) were prospectively studied. All had compromised image quality, 20 (39%) had arrhythmias, and 10 (20%) had pacemakers. RTP was performed at rest and during BSE using Definity and Contrast Pulse Sequencing, with assessment of Doppler pressure gradients. Diagnoses included tetralogy of Fallot (n = 27), transposition of the great arteries (TGA) atrial switch (n = 10), TGA arterial switch (n = 2), aortic valve disease (n = 4), Fontan (n = 4), and Kawasaki disease (n = 4). UC with RTP improved endocardial border definition, with increased number of left ventricular (LV) and right ventricular (RV) segments visualized at rest (P < 0.0001) and during stress. LV ejection fraction (EF) and RV fractional area change (FAC) were measurable at rest and peak stress, RV FAC correlating closely with same-day magnetic resonance EFs (r = 0.72; P < 0.001). UC enhanced Doppler signals, enabling subpulmonary ventricular systolic pressure measurements at rest and stress. In six patients, marked elevations of subpulmonary ventricular systolic pressure were detected with UC during BSE, and quantifiable ventricular dysfunction. No adverse events occurred, other than transient low back pain in one patient. CONCLUSION: UC at rest and with supine BSE enables safe and comprehensive assessment of anatomy, haemodynamics, and biventricular functional and perfusion reserve in adolescents and young adults with surgically modified CHD.

Sonothrombolysis of intra-catheter aged venous thrombi using microbubble enhancement and guided three-dimensional ultrasound pulses.

BACKGROUND: Central venous and arterial catheters are a major source of thromboembolic disease in children. The investigators hypothesized that guided high-mechanical index (MI) impulses from diagnostic three-dimensional (3D) ultrasound during an intravenous microbubble infusion could dissolve these thrombi. METHODS: An in vitro system simulating intracatheter thrombi was created and then treated with guided high-MI impulses from 3D ultrasound, using low-MI microbubble sensitive imaging pulse sequence schemes to detect the microbubbles. Ten aged thrombi >24 hours old were tested using 3D ultrasound coupled with a continuous diluted microbubble infusion (group A) and 10 with 3D ultrasound alone (group B). RESULTS: The mean thrombus age was 28.6 hours (range, 26.6-30.3 hours). Group A exhibited a 55 +/- 19% reduction in venous thrombus size compared with 31 +/- 10% in group B (P = .008). Feasibility testing was performed in four pigs, establishing an in vivo model to investigate further the efficacy of this approach. CONCLUSIONS: Sonothrombolysis of aged intracatheter venous thrombi can be achieved with commercially available microbubbles and guided high-MI ultrasound from a diagnostic 3D transducer.

Is combined atrial volumetrics by two-dimensional echocardiography a suitable measure for quantitative assessment of the hemodynamic significance of patent ductus arteriosus in neonates and infants?

BACKGROUND: Patent ductus arteriosus (PDA) is a common cardiac problem in neonates and infants, but determination of its hemodynamic significance can be challenging. We hypothesized that combined left (LA) and right atrial (RA) volumes physiologically best reflect hemodynamically significant patent ductus arteriosus (HSPDA), and utilized two-dimensional echocardiography (2DE) derived atrial volumes to test this hypothesis. METHODS: 2DE examinations with good-quality images in 138 neonates <3 months corrected gestational age with PDA, and 50 normal neonates without PDA were selected. Measurements of LA, RA, and combined atrial volumes were performed, in addition to transductal diameters, left atrial to aortic dimension (LA:Ao), and left ventricular end-diastolic to aortic dimension ratios. An experienced cardiologist, blinded to 2DE images of atria and ventricles and to the above measurements, independently assessed HSPDA based only on images and Doppler data of the ductus itself, thus identifying each PDA as of low hemodynamic significance or HSPDA. RESULTS: Receiver operating characteristic (ROC) curves showed indexed LA volumes and LA/RA volume ratios to have moderate power to discriminate HSPDA from low hemodynamic burden PDA. Classic LA:Ao ratio, combined atrial volumes, and RA volumes yielded ROC areas that appeared less promising as discriminators for HSPDA. CONCLUSION: Atrial volume measurements in neonates and infants have a linear association with body surface area and show acceptable inter- and intraobserver agreement. Indexed LA volume and LA/RA volume ratio are potentially useful markers for HSPDA. RA dilation due to left to right shunting through the patent foramen ovale as quantified by RA volume measurements does not appear to be an important marker for HSPDA.

Coarctation of a right-sided aortic arch and an unusual vascular ring.

The clinical presentation, diagnostic workup, and surgical therapy of a coarcted right-sided aortic arch associated with a vascular ring are described.