Prenatal Diagnosis of a Ductal-Dependent Branch Pulmonary Artery: Extra Vessels in the 3-Vessel and Trachea View.

Obtaining a three-vessel (3V) and three-vessel and trachea (3VT) view from the fetal upper mediastinum is now considered to be part of standard imaging protocol for routine obstetric cardiac screening examinations. We report two fetal cases of an anomalous pulmonary artery origin, utilizing the standard 3V and 3VT views. Further imaging led to a rare diagnosis of bilateral ductus arteriosus with discontinuous branch pulmonary arteries in the absence of any other congenital heart defect. We briefly discuss the imaging features, differential diagnoses, and management of this rare entity.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

BACKGROUND: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria. OBJECTIVES: The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. METHODS: CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. RESULTS: Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. CONCLUSIONS: CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

Large single centre experience with the Cera™ and CeraFlex™ occluders for closure of interatrial communications: usefulness of the flexible rotation feature.

We present our experience with the Cera (CO) and the CeraFlex occluder (CFO) in transcatheter closure of interatrial communications (IAC). Between 2013 and 2016, 201 patients (75 males, 16 with patent foramen ovale), aged 27 ± 19 (5-75) years, underwent percutaneous closure of IAC using CO or CFO in our institution. After transoesophageal imaging, the procedure was aborted in 7 young paediatric (6-13 years old) patients (3 multiple holes, 3 too small septum, 1 leak with 38 mm occlusion balloon). The occluder was removed prior to release in 11 patients (5.7%), while occlusion was successful in 183 patients (94.3%) with 44 CO, 136 CFO, and 3 Cera multifenestrated occluders. There were no deaths, embolizations, or major complications. Small residual shunts were demonstrated in 8 patients immediately after implantation, 4 (8.5%) with CO and 4 (2.9%) with CFO, all disappearing after 3 months. Over 1.8 ± 1.7 year follow-up, all patients improved with 2 asymptomatic, transient pericardial effusions and 5 adults with transient supraventricular arrhythmias, treated medically for 6 months. IAC closure with CO and CFO proved safe with favourable success rates and few, nonserious complications. The CFO flexible rotation feature helped in conforming to various septal anatomies, minimising manoeuvres and possibly post-occlusion leaks.

Complete heart block complicating the acute phase of respiratory syncytial virus bronchiolitis.

Respiratory syncytial virus is the most common pathogen causing lower respiratory tract infection in infants. In recent years, the importance of extrapulmonary complications and longterm sequelae including those involving the cardiovascular system has become apparent. We report on a 10 month old infant with respiratory syncytial virus bronchiolitis, the acute phase of which was complicated by irreversible complete heart block, which was managed conservatively.

Aorto-Right Ventricular Tunnel: An Uncommon Problem with a Common Solution.

Aorto-ventricular tunnel is a rare congenital malformation, and aorto-right ventricular tunnel (ARVT) is an even less common entity. Here, we report the case of a 3-month-old female who underwent successful surgical closure of ARVT. The origin of the right coronary artery was proximal to the ostium of the tunnel.

Right ventricular myxoma in a patient with tetralogy of Fallot.

INTRODUCTION: Cardiac myxoma is the most common primary cardiac tumour in adulthood and may present in the context of Carney's complex. PRESENTATION OF CASE: A 32-year-old male with a history of repaired tetralogy of Fallot in childhood was admitted with severe pulmonary valve regurgitation and a mobile mass in the right ventricle. The patient underwent pulmonary valve replacement and mass excision. Pathology examination showed myxoma. DISCUSSION: In the majority of cases myxomas originate in the atria, nevertheless they can also be found in a ventricular cavity. Myxoma is a prevalent feature of Carney's complex, an inherited, autosomal disease, characterised by multiple tumours in several organs. Tetralogy of Fallot has also been described in association with Carney's complex. CONCLUSION: Coexistence of tetralogy of Fallot with a cardiac ventricular myxoma in a patient not affected from Carney's complex or other familial syndrome.

Saw-tooth cardiomyopathy.

We present an unusual case of cardiomyopathy in a two month old male infant with a grade-I systolic murmur. Echocardiographic examination disclosed left ventricular (LV), dysplasia with saw-tooth like inwards myocardial projections extending from the lateral walls towards the LV cavity. There was mild LV systolic dysfunction with apical hypokinesia. Cardiovascular magnetic resonance demonstrated in detail these cross bridging muscular projections originating from the inferior interventricular septum and lateral LV wall, along with areas of hypokinesis at the LV septum and apex in a noncoronary distribution, without any late gadolinium enhancement. We have termed this condition saw-tooth cardiomyopathy because of the very characteristic appearance.

Fatal cardiac arrest due to an unusual fibroma of the pulmonary valve.

Fibroma, the second most common tumor in childhood, usually is a large solitary mass involving the ventricular septum or the left and rarely the right free ventricular wall. An unusual case of a 5.2-year-old boy with a large mass originating from the pulmonary valve is reported. The patient presented for evaluation of a murmur. Echocardiography and cardiac magnetic resonance imaging showed a large tumor extending from the distal right ventricular outflow tract through the pulmonary valve and into the main pulmonary artery causing moderate obstruction. The patient had a fatal cardiac arrest while awaiting surgery. The postmortem examination proved the tumor to be a fibroma.

Transcatheter closure of coronary arterial fistulas using the new Amplatzer vascular plug.

We report our initial experience in using the Amplatzer vascular plug for closure of coronary arterial fistulas. The self-expanding, cylindrical, device is made from Nitinol wire mesh, and is available from 4 to 16 millimetres in diameter. We have now used the device to close fistulas in 3 patients, aged from 3 to 14 years, who presented with ratios of pulmonary-to-systemic flow from 1.5 to 3. In 2 patients, fistulas arising from the proximal right and left coronary arteries, with maximal diameters of 9 and 10 millimetres, respectively, had their narrowest diameter, of 6 millimetres, proximal to the entrance into the right atrium via a saccular aneurysm. The third fistula, with a maximal diameter of 16 millimetres, and with its origin from the circumflex coronary artery, entered the right atrium with nearly unrestricted flow, its narrowest diameter being 8 millimetres. For interventional closure, we chose plugs twice the diameter of the narrowest segment of the fistula, thus using 2 devices of 12 millimetres and one of 16 millimetres diameter. An arteriovenous loop was established through the fistula by snaring an exchange guide wire. Using a 7 or 8 French guide catheter inserted through the femoral vein, all plugs were placed at the narrowest segment of the fistula, leading to immediate complete closure of 2 fistulas. The third patient, with a fistula of the circumflex coronary artery, who received the largest plug initially had residual flow, but the fistula was found to be completely occluded at 12 months follow-up examination. We have demonstrated, therefore, safe and effective usage of the new vascular plug for transcatheter closure of moderate- to large-sized coronary arterial fistulas. The plug offers an alternative to cardiac surgery, or occlusion using coils.

Overnight change in brain natriuretic peptide levels in children with sleep-disordered breathing.

STUDY OBJECTIVES: Obstructive sleep-disordered breathing is accompanied by episodic increases in left ventricle afterload due to large negative swings in intrathoracic pressure and repetitive surges in arterial pressure. Brain natriuretic peptide (BNP) is released by ventricular myocytes in response to pressure and volume overload. It was hypothesized that in children with snoring, overnight change in BNP levels is correlated with severity of disturbance in respiration. DESIGN: Evening and morning plasma levels of BNP were measured in children with snoring referred for polysomnography. SETTING: A sleep disorders laboratory in a university hospital. PARTICIPANTS: Twenty-two children with apnea-hypopnea index (AHI) > or = 5/h (mean +/- SD age, 6.4 +/- 2.5 years), 60 children with AHI < 5/h (mean age, 7 +/- 2.9 years), and 27 control subjects without snoring (mean age, 7.8 +/- 3.7 years) were recruited. MEASUREMENTS AND RESULTS: Overnight change in BNP (log-transformed ratio of morning-to-evening levels) was larger in children with AHI > or = 5/h, compared to those with AHI < 5/h or to control subjects (0.1 +/- 0.19 vs 0.01 +/- 0.14 vs - 0.06 +/- 0.18; p < 0.05). Children with AHI > or = 5/h had an odds ratio of 4.33 (95% confidence interval, 1.34 to 14) for change in peptide levels > 0.15 relatively to subjects with AHI < 5/h. AHI and oxygen saturation of hemoglobin nadir were significant predictors of overnight change in peptide levels. CONCLUSIONS: In children with snoring, overnight increase in BNP levels is correlated with severity of disturbance in respiration during sleep, which may indicate presence of nocturnal cardiac strain.

Nonfluoroscopic catheter navigation for radiofrequency catheter ablation of supraventricular tachycardia in children.

BACKGROUND: Radiofrequency catheter ablation (RCA) of supraventricular tachycardia (SVT) in children is highly successful but requires exposure to radiation. Nonfluoroscopic mapping systems may significantly reduce fluoroscopy time. METHODS: Forty consecutive pediatric patients who underwent RCA for accessory pathways (AP) or AV nodal reentrant tachycardia (AVNRT) with use of a nonfluoroscopic navigation system (Ensite NavX) (group A) were compared retrospectively to 40 consecutive patients with similar diagnoses who underwent RCA with fluoroscopic guidance only (group B). RESULTS: Group A (mean age 12.1+/-2.9 years, mean weight 47+/-13.9 kg) consisted of 11 patients (27.7%) with AVNRT and 29 (72.5%) with AP. Group B (mean age 10.9+/-3.1 years, mean weight 47.1+/-17.1 kg) consisted of 7 patients (17.5%) with AVNRT and 33 (82.5%) with AP. There were no significant differences in AP location, patients with congenital heart disease, and number of radiofrequency lesions. Fluoroscopy time was significantly shorter in group A than in group B (10.4+/-6.1, range 3.1-28.8 minutes, vs 24.9+/-16.0, range 4.4-82.0 minutes, P<0.0001). Procedure duration was also significantly shorter in group A than in group B (170+/-68.5, range 90-420 minutes, vs 218+/-69.3, range 90-360 minutes, P<0.0001). Initial success was 95% in group A and 100% in group B. Tachycardia recurrences occurred in two patients in group A (5%) and six patients in group B (15%). Final success, including repeat ablations for recurrences or failures, was 100% in both groups. CONCLUSIONS: The use of a nonfluoroscopic system for catheter navigation significantly reduced fluoroscopy exposure and total procedure duration of RCA of common SVT substrates in children.

Cardiovascular magnetic resonance evaluation of patients with transposition of the great arteries following atrial switch surgical correction.

We present a method of imaging patients with surgically corrected transposition of the great arteries using cardiac magnetic resonance imaging and we describe the findings from a typical case. Cardiac magnetic resonance is superior to other imaging methods for patients with complex congenital heart diseases, both for establishing the initial diagnosis and for subsequent follow-up after surgical therapy.

Diverse experience with the CardioSEAL and STARFlex septal occluders.

Apart from closure of atrial septal defects, there is little information concerning the use of the CardioSEAL family of occluders in congenitally malformed hearts. We review here our initial experience using the CardioSEAL and STARFlex occluders in 12 patients aged 17.3 +/- 11.2 years, with a range from 4 to 34 years. Of the patients, 5 had fenestrated extracardiac Fontan procedures, 5 had persistent patency of the arterial duct, 1 had a leak across a Mustard baffle, and the final patient had a huge pulmonary arteriovenous malformation. We implanted successfully 9 CardioSEAL, and 3 STARFlex occluders, with sizes from 17 to 40 mm. In one patient, the occluder embolized to the right pulmonary artery, from where it was retrieved through the catheter. In two patients, there was a trivial residual leak immediately after implantation, but no patient had a residual leak after 6 months of follow-up. We noted improved ventricular dimensions, without any fractures of the arms of the occluders, perforations, or disturbances of flow after 2.4 +/- 0.9 years of follow-up. We have demonstrated, therefore, the versatility of the CardioSEAL and STARFlex occluders, which have been used safely and effectively to close a variety of intra and extracardiac communications other than atrial septal defects.