Anomalous Left Coronary Artery Connected to the Pulmonary Artery in a 15-Year-Old Girl: Case Report and Discussion on Secondary Prevention of Sudden Death.

Background. Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) is a rare congenital heart disease. Adaptive development of sufficient heterocoronary collaterality in the newborn may allow survival to a later age. In older children or adults, malignant ventricular arrhythmias can reveal the disease. Case Report. A 15-year-old girl was referred to the local hospital after a resuscitated out-of-hospital cardiac arrest. CT scan and coronary angiography revealed an ALCAPA. Direct aortic reimplantation of the left coronary artery was performed. Postoperative ECG monitoring showed short episodes of nonsustained ventricular tachycardia. Transthoracic echocardiography and cardiac MRI revealed subendocardial fibrosis of the anterolateral papillary muscle. Beta-blockade therapy was initiated at first intention. After hospital discharge, the patient reported several fainting without loss of consciousness. Considering sudden death nonrelated to effort, episodes of nonsustained ventricular tachycardia, and areas of myocardial fibrosis, the patient underwent subcutaneous cardioverter-defibrillator implantation. 6-month follow-up is satisfactory without clinical or rhythmic abnormalities. Discussion. Indication for surgical correction of ALCAPA is well defined, but rhythmic secondary prevention after resuscitated cardiac arrest is less consensual. Cardiac MRI is an essential tool in the identification of a potential rhythmic substrate and should be taken into account in the discussion of a preventive cardioverter-defibrillator implantation.

Position paper concerning the competence, performance and environment required for the practice of ablation in children and in congenital heart disease.

The population of patients with congenital heart disease (CHD) is continuously increasing, and a significant proportion of these patients will experience arrhythmias because of the underlying congenital heart defect itself or as a consequence of interventional or surgical treatment. Arrhythmias are a leading cause of mortality, morbidity and impaired quality of life in adults with CHD. Arrhythmias may also occur in children with or without CHD. In light of the unique issues, challenges and considerations involved in managing arrhythmias in this growing, ageing and heterogeneous patient population and in children, it appears both timely and essential to critically appraise and synthesize optimal treatment strategies. The introduction of catheter ablation techniques has greatly improved the treatment of cardiac arrhythmias. However, catheter ablation in adults or children with CHD and in children without CHD is more technically demanding, potentially causing various complications, and thus requires a high level of expertise to maximize success rates and minimize complication rates. As French recommendations regarding required technical competence and equipment are lacking in this situation, the Working Group of Pacing and Electrophysiology of the French Society of Cardiology and the Affiliate Group of Paediatric and Adult Congenital Cardiology have decided to produce a common position paper compiled from expert opinions from cardiac electrophysiology and paediatric cardiology. The paper details the features of an interventional cardiac electrophysiology centre that are required for ablation procedures in adults with CHD and in children, the importance of being able to diagnose, monitor and manage complications associated with ablations in these patients and the supplemental hospital-based resources required, such as anaesthesia, surgical back-up, intensive care, haemodynamic assistance and imaging. Lastly, the need for quality evaluations and French registries of ablations in these populations is discussed. The purpose of this consensus statement is therefore to define optimal conditions for the delivery of invasive care regarding ablation of arrhythmias in adults with CHD and in children, and to provide expert and - when possible - evidence-based recommendations on best practice for catheter-based ablation procedures in these specific populations.

Adenosine plasma level in patients with paroxysmal or persistent atrial fibrillation and normal heart during ablation procedure and/or cardioversion.

The mechanism of atrial fibrillation (AF) in patients with normal heart remains unclear. While exogenous adenosine can trigger AF, nothing is known about the behavior of endogenous adenosine plasma level (APL) at the onset of AF and during ablation procedure. Ninety-one patients (68 with paroxysmal AF: 40 males, 66 ± 16 years; 23 with persistent AF: 14 males, 69 ± 11 years) and 18 controls were included. Among paroxysmal patients: i) medical therapy alone was performed in 45 cases and ablation procedure in 23. AF was spontaneously resolutive in 6 cases; ii) 23 underwent ablation procedure and blood was collected simultaneously in a brachial vein and in the left atrium; 17 were spontaneously in sinus rhythm while 6 were in sinus rhythm after direct current cardioversion. Among persistent patients: i) in 17 patients, blood samples were collected in a brachial vein before and after direct current cardioversion; ii) in 6 patients, blood samples were collected simultaneously in a brachial vein and in left atrium before and after cardioversion during ablation procedure. CV-APL was higher in patients with persistent AF vs patients with paroxysmal AF (median [range]: 0.9[0.6-1.1] vs 0.7[0.4-1.1] µM; p < 0.001). In patients with paroxysmal AF, LA-APL increased during the AF episode (0.95[0.85-1.4] vs 2.7[1.5-7] µM; p = 0.03) and normalized in sinus rhythm after DCCV. In patients with persistent AF, LA-APL was higher than CV-APL (1.2[0.7-1.8] vs 0.9[0.6-1.1] µM; p < 0.001), and both normalized in sinus rhythm (CV-APL: 0.8[0.6-1.1] vs 0.75[0.4-1] µM; p = 0.03), (LA-APL: 1.95[1.3-3] vs 1[0.5-1.15] µM; p = 0.03). The occurrence of AF is associated with a strong increase of APL in the atrium. The cause of this increase needs further investigations.

Theophylline as an adjunct to control malignant ventricular arrhythmia associated with early repolarization.

Early repolarization (ER) has been associated with an increased risk of sudden cardiac arrest. Interestingly, ventricular arrhythmias seem to be triggered by parasympathetic stimulation. In the present case report, we describe complete control of highly frequent malignant ventricular arrhythmias after adding theophylline to ineffective oral hydroquinidine and high-rate pacing in a patient suffering from malignant ER. We hypothesize that the theophylline-mediated enhanced beta-adrenergic stimulation could reduce the transmural myocardial voltage discrepancy by increasing the inward ICa,L current.

Vagal Reactions during Cryoballoon-Based Pulmonary Vein Isolation: A Clue for Autonomic Nervous System Modulation?

Although paroxysmal atrial fibrillation (AF) is known to be initiated by rapid firing of pulmonary veins (PV) and non-PV triggers, the crucial role of cardiac autonomic nervous system (ANS) in the initiation and maintenance of AF has long been appreciated in both experimental and clinical studies. The cardiac intrinsic ANS is composed of ganglionated plexi (GPs), located close to the left atrium-pulmonary vein junctions and a vast network of interconnecting neurons. Ablation strategies aiming for complete PV isolation (PVI) remain the cornerstone of AF ablation procedures. However, several observational studies and few randomized studies have suggested that GP ablation, as an adjunctive strategy, might achieve better clinical outcomes in patients undergoing radiofrequency-based PVI for both paroxysmal and nonparoxysmal AF. In these patients, vagal reactions (VR) such as vagally mediated bradycardia or asystole are thought to reflect intrinsic cardiac ANS modulation and/or denervation. Vagal reactions occurring during cryoballoon- (CB-) based PVI have been previously reported; however, little is known on resulting ANS modulation and/or prevalence and significance of vagal reactions during PVI with the CB technique. We conducted a review of prevalence, putative mechanisms, and significance of VR during CB-based PVI.

Electromyographic monitoring for prevention of phrenic nerve palsy in second-generation cryoballoon procedures.

BACKGROUND: Electromyography-guided phrenic nerve (PN) monitoring using a catheter positioned in a hepatic vein can aid in preventing phrenic nerve palsy (PNP) during cryoballoon ablation for atrial fibrillation. We wanted to evaluate the feasibility and efficacy of PN monitoring during procedures using second-generation cryoballoons. METHODS AND RESULTS: This study included 140 patients (43 women) in whom pulmonary vein isolation was performed using a second-generation cryoballoon. Electromyography-guided PN monitoring was performed by pacing the right PN at 60 per minute and recording diaphragmatic compound motor action potential (CMAP) via a quadripolar catheter positioned in a hepatic vein. If a 30% decrease in CMAP amplitude was observed, cryoapplication was discontinued with forced deflation to avoid a PNP. Monitoring was unfeasible in 8 of 140 patients (5.7%), PNP occurred in 1. Stable CMAP amplitudes were achieved before ablation in 132 of 140 patients (94.3%). In 18 of 132 patients (13.6%), a 30% decrease in CMAP amplitude occurred and cryoablation was discontinued. Each time, recovery of CMAP amplitude took <60 s. In 9 of 18 cases, a second cryoapplication in the same pulmonary vein was safely performed. We observed no PNP or complication related to electromyography-guided PN monitoring. CONCLUSIONS: Electromyography-guided PN monitoring using a catheter positioned in a hepatic vein seems feasible and effective to prevent PNP during cryoballoon ablation using second-generation cryoballoon.

Novel electromyographic monitoring technique for prevention of right phrenic nerve palsy during cryoballoon ablation.

BACKGROUND: Right phrenic nerve palsy (PNP) is the most frequent complication of cryoballoon ablation. Diaphragmatic electromyography can predict PNP with a comfortable safety margin. Our goal was to evaluate the feasibility, efficacy, and safety of electromyography-guided PN monitoring using a novel hepatic vein approach for prevention of PNP. METHODS AND RESULTS: This study includes 57 patients (47 males) indicated for cryoballoon ablation for treatment of atrial fibrillation. During right superior pulmonary vein ablation, the PN was paced at 60 beats per minute and diaphragmatic compound motor action potential (CMAP) amplitude was recorded via a quadripolar catheter positioned in a subdiaphragmatic hepatic vein. If a 30% drop in CMAP amplitude was observed, ablation was discontinued with forced deflation. Reliable recording of CMAP before ablation was feasible in 50 of 57 patients (88%). In 7 patients (12%), stable PN pacing could not be achieved. In 44 of 50 patients, CMAP amplitude remained constant during cryoapplication. The mean value of CMAP amplitude was 639.7±240.5 µV; mean variation was 13±4.3%. In 6 of 50 patients (12%) including 5 treated with a 23-mm cryoballoon and 1 with a 28-mm cryoballoon, the 30% reduction cutoff was reached and cryoablation was discontinued. Recovery of CMAP amplitude after discontinuing cryoablation took <60 seconds in all cases. No PNP or complication related to PN monitoring occurred. CONCLUSIONS: Recording of diaphragmatic CMAP using a catheter positioned in a subdiaphragmatic hepatic vein seems feasible during cryoballoon ablation. Electromyography-guided PN monitoring seems safe and potentially helpful for prevention of PNP.

Histopathological effects and evolution of transvenous ß-radiation applications in right and left atria: an animal study.

AIMS: ß-radiation is a novel potential energy source for the creation of myocardial lesions. While the feasibility of delivering ß-radiation via a deflectable transvenous catheter has been described, dose effects and the time course of histopathological changes have not been previously assessed. The purpose of this study was to characterize pathological aspects of cardiac lesions induced by ß-radiation in an animal model at various stages of evolution and in response to different dose exposures. METHODS AND RESULTS: Nine dogs and one pig were studied. The cavotricuspid isthmus, antrum of pulmonary veins (PVs), and mitral isthmus were irradiated (25, 50, 75, or 100 Gy) with strontium-yttrium-90, delivered via a deflectable catheter (cavotricuspid isthmus and mitral isthmus) or a double-loop catheter (antrum of PVs). Eighteen lesions were created. Animals were sacrificed at 2 weeks, 1 month, 3 months, or 6 months. Lesions were processed for morphometric histopathological analyses. Over the first month, lesions were characterized by inflammation, haemorrhage, and myocyte necrosis. Thereafter, fibrotic replacement was predominant. Transmurality of lesions was observed in 50% of cases, with no dose-response effect (P = 0.976). Surface fibrin thrombus was present in 50% of cases and was essentially limited to lesions assessed within the first month. No neighbouring injury or pulmonary venous stenosis was observed. CONCLUSIONS: Atrial lesions created by ß-radiation are characterized by an inflammatory phase with surface fibrin thrombosis during the first month and replacement fibrosis thereafter. No appreciable dose-response effect was noted within the 25-100 Gy range tested.

Diaphragmatic electromyography during cryoballoon ablation: a novel concept in the prevention of phrenic nerve palsy.

BACKGROUND: Hemidiaphragmatic paralysis is the most frequent complication associated with cryoballoon ablation for atrial fibrillation. To date, no preventive strategy has proved effective. OBJECTIVE: We sought to assess the feasibility of diaphragmatic electromyography during cryoballoon ablation, explore the relationship between altered signals and phrenic nerve palsy, and define characteristic changes that herald hemidiaphragmatic paralysis. METHODS: Cryoballoon ablation was performed in the right superior pulmonary vein or superior vena cava in 16 mongrel dogs weighing 37.7 ± 2.4 kg, at sites determined by phrenic nerve capture. During ablation, the phrenic nerve was paced at 60 bpm from the superior vena cava while recording diaphragmatic compound motor action potentials (CMAPs) by esophageal decapolar catheters. Diaphragmatic excursion was monitored by fluoroscopy and abdominal palpation. RESULTS: Before ablation, the CMAP amplitude was 592 (interquartile range 504, 566) µV, initial latency 21.5 ± 4.2 ms, peak latency 64.7 ± 21.1 ms, and duration 101.7 ± 13.3 ms. Hemidiaphragmatic paralysis was obtained in all dogs 62 ± 34 seconds into the cryoapplication. The CMAP amplitude decreased exponentially, with no patterned changes in latencies and duration. Discriminatory analyses by receiver-operating curve characteristics identified a 30% reduction in CMAP amplitude as the most predictive cutoff value for hemidiaphragmatic paralysis (c-statistic 0.965; P<.0001). This criterion presaged diaphragmatic paralysis, as detected by abdominal palpation, by 31 ± 23 seconds. CONCLUSION: Diaphragmatic electromyographic signals could be reliably recorded during cryoballoon ablation. An exponential decrease in CMAP amplitude precedes diaphragmatic paralysis, with a 30% reduction yielding the best discriminatory potential. A promising safety margin was detected, which merits prospective validation.