Multiple QRS Morphologies: Findings on an Initial Newborn ECG.

Regular wide complex tachycardia carries with it a standard array of differential diagnoses. This electrocardiogram demonstrates wide complex tachycardia and multiple QRS configurations in a neonate without structural heart disease with an uncommon suspected underlying diagnosis. (Level of Difficulty: Advanced.).

Heart Failure as the Initial Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery.

Coronary arteries arising from the pulmonary artery have an incidence of 0.002% in the general population. We present a 29-year-old woman who presented to our hospital with acute decompensated heart failure and atrial fibrillation with a rapid ventricular rate. She underwent a cardiac catheterization to rule out ischemic disease, which revealed retrograde contrast flow through the left coronary artery from the right coronary artery. A coronary computed tomography (CT) angiogram was pursued which showed the presence of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA). For the management of her atrial fibrillation, she was electrically cardioverted. She was discharged on guideline-directed medical therapy for her heart failure, with a cardiac surgery referral for the surgical fixation of her ALCAPA.

Long-term outcomes up to 25 years following balloon pulmonary valvuloplasty: A multicenter study.

OBJECTIVE: Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS). BACKGROUND: Long-term data following BPV is limited to small, single center studies. METHODS: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis. RESULTS: Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mm Hg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had ≥ moderate PI, 41 patients (17%) had residual PS > 40 mm Hg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with ≥ moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention. CONCLUSION: Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV.

Transseptal catheter decompression of the left ventricle during extracorporeal membrane oxygenation.

Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) can result in left-ventricular distension and the development of pulmonary edema. We present the case of a 13-year-old girl who presented with cardiogenic shock. VA-ECMO was initiated, but after 6 days, severe left-ventricular distension resulted in decreased VA-ECMO flows. With guidance by bedside transesophageal echocardiography, a percutaneous atrial transseptal cannula was placed and connected to the venous circuit, thus decompressing the left ventricle. The patient improved, was weaned from VA-ECMO 5 days later, and was discharged from the hospital. Bedside transseptal catheter insertion is an effective method of left-ventricular decompression.

Prospective assessment after pediatric cardiac ablation: fate of intracardiac structure and function, as assessed by serial echocardiography.

BACKGROUND: Catheter ablation puts cardiac valves at risk of damage, and children are of particular concern. METHODS: A multicenter prospective study was performed to assess the results and risks associated with radiofrequency (RF) ablation in children. Patients were aged 0 to 16 years with supraventricular tachycardia due to accessory pathway or atrioventricular node reentry, excluding patients with more than trivial congenital heart disease. A total of 481 patients were recruited into the prospective cohort and were followed up at 2, 6 and, 12 months after ablation. Complete echocardiograms were obtained before and at intervals after RF ablation, and they were reviewed by experts who were blinded with respect to diagnosis, outcome, pathway site, and study interval. RESULTS: Moderate valve insufficiency was quite rare (0.12%), but mild insufficiency was common, both before ablation (42.43%) and at 2 months after ablation (40.49%). Analysis of paired readings failed to demonstrate a tendency toward increased insufficiency of valves adjacent to ablation targets, with the exception of the tricuspid valve after the ablation of right freewall pathways and atrioventricular node reentry, but the degree of change was small. No clear changes in left ventricular systolic or diastolic function were observed, and dyskinesis was rare and not related to the ablation target. No intracardiac thrombosis was observed. CONCLUSIONS: Serious injury to cardiac valves due to RF ablation is very rare, but the tricuspid valve may be mildly affected in some cases. We identified no clear effect of RF ablation on cardiac wall motion or on left ventricular function.

New approach to the surgical management of pulmonary arteriovenous malformations after cavopulmonary anastomosis.

The development of pulmonary arteriovenous malformations after cavopulmonary bypass in patients with congenital heart disease is well documented. We report successful management of pulmonary arteriovenous malformations after cavopulmonary bypass in a patient with an interrupted inferior vena cava (IVC) and multiple hepatic veins utilizing an extracardiac conduit from the hepatic veins to the hemiazygous continuation of the interrupted IVC. This technique, performed without circulatory arrest or an atriotomy, may limit morbidity associated with intracardiac procedures in patients with single ventricle morphology. Furthermore, this case suggests an alternative technique for completion Fontan in patients with an interrupted IVC and multiple hepatic venous drainage.

Prospective assessment after pediatric cardiac ablation: design and implementation of the multicenter study.

A multicenter prospective study was designed and implemented as an activity of the Pediatric Electrophysiology Society to assess the risks associated with radiofrequency ablation in children. Patients (age 0-15 years) with supraventricular tachycardia due to accessory pathways or atrioventricular nodal reentry were enrolled and studied prior to ablation and periodically by clinical evaluation, electrocardiogram (ECG), Holter monitor, and echocardiogram. In addition, a national registry was established, to which the contributing centers report all pediatric patients undergoing ablation at their center. Initial electrophysiological study tracings and all noninvasive studies undergo blinded outside review for quality control. Clinical endpoints were death, recurrence, proarrhythmia, and echocardiographic abnormality. A pilot study demonstrated excellent agreement concerning diagnoses of previously reported ablation patients between the reporting center and the blinded reviewer (kappa = 0.938 +/- 0.062). A total of 317 patients were enrolled in the ongoing study from April 1, 1999 to December 31, 2000. The success rate of ablations was 96% with a complication rate of 4.3% for electrophysiological study and 2.9% for the ablation procedure. Comparison of the registry group versus the study group shows that the groups are comparable in terms of patient characteristics, diagnoses, and the results of ablation making it less likely that the sample of prospectively enrolled patients is biased.