Jeffrey P. Moak

Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela.

OBJECTIVE We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects. METHODS A multi-institutional study was performed to identify common clinical features and possible risk factors associated with late-onset dilated cardiomyopathy in patients born with congenital CHB. RESULTS Congenital heart block was diagnosed in utero in 12 patients and at birth in four patients. Ten of 16 patients had serologic findings consistent with neonatal lupus syndrome (NLS). A pericardial effusion was evident on fetal ultrasound in six patients. In utero determination of left ventricular (LV) function was normal in all. Following birth, one infant exhibited a rash consistent with NLS and two had elevated hepatic transaminases and transient thrombocytopenia. In the early postnatal period, LV function was normal in 15 patients (shortening fraction [SF] = 34 +/- 7%) and was decreased in one (SF = 20%). A cardiac pacemaker was implanted during the first two weeks of life in 15 patients and at seven months in one patient. Left ventricular function significantly decreased during follow-up (14 days to 9.3 years, SF = 9% +/- 5%). Twelve of 16 patients developed congestive heart failure before age 24 months. Myocardial biopsy revealed hypertrophy in 11 patients, interstitial fibrosis in 11 patients, and myocyte degeneration in two patients. Clinical status during follow-up was guarded: four patients died from congestive heart failure; seven required cardiac transplantation; one was awaiting cardiac transplantation; and four exhibited recovery of SF (31 +/- 2%). CONCLUSIONS Despite early institution of cardiac pacing, some infants with CHB develop LV cardiomyopathy. Patients with CHB require close follow-up not only of their cardiac rate and rhythm, but also ventricular function.

2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia, and Vasovagal Syncope

Robert S. Sheldon, Blair P. Grubb II, Brian Olshansky, Win-Kuang Shen, Hugh Calkins, Michele Brignole, Satish R. Raj, Andrew D. Krahn, Carlos A. Morillo, Julian M. Stewart, Richard Sutton, Paola Sandroni, Karen J. Friday, Denise Tessariol Hachul, Mitchell I. Cohen, Dennis H. Lau, Kenneth A. Mayuga, Jeffrey P. Moak, Roopinder K. Sandhu, Khalil Kanjwal

Mortality Following Radiofrequency Catheter Ablation (from the Pediatric Radiofrequency Ablation Registry)

Deaths have been reported following radiofrequency catheter ablation (RFCA), but the mortality rate in children has not been defined. This study sought to analyze the incidence and the factors associated with mortality related to RFCA. Ten of 4,651 cases (0.22%) reported to the Pediatric RFCA Registry resulting in death were reviewed and compared with a matched control group (n = 18). Death occurred in 5 of 4,092 children (0.12%, ages 0.1 to 13.3 years) with structurally normal hearts. Death was related to traumatic injury, myocardial perforation and hemopericardium, coronary or cerebral thromboembolism, and ventricular arrhythmia. All cases were left-sided (p = 0.019 vs right or septal) supraventricular arrhythmias with radiofrequency applications in the systemic atrium and/or ventricle, and all procedures were successful. Mortality occurred in 5 of 559 children (0.89%, p = 0.001 vs normals, ages 1.5 to 17.4 years) with structural heart disease. No new pathology except the mural radiofrequency lesions was seen at autopsy. Those with structurally normal hearts who died were smaller (32.7 vs 55.6 kg, p = 0.023) and had more radiofrequency applications (26.3 vs 8.7, p = 0.019) than those who survived. No differences were demonstrated for those with abnormal hearts. Operator experience was not different (deaths 103 +/- 106 vs controls 117 +/- 125, p = 0.41). Mortality associated with pediatric RFCA is rare, but is more frequent when there is underlying heart disease, lower patient weight, greater number of radiofrequency energy applications, and left-sided procedures. Operator experience does not appear to be a factor leading to mortality.

Case report: pulmonary vein stenosis following RF ablation of paroxysmal atrial fibrillation: successful treatment with balloon dilation.

Paroxysmal atrial fibrillation and atrial tachycardia may originate from a focal source in one or multiple pulmonary veins. A focal origin facilitates a potential cure amendable to radiofrequency ablation. Herein we report the case of a 16 year old adolescent male with a tachycardia induced cardiomyopathy who presented with very frequent paroxysmal episodes of atrial fibrillation, atrial flutter and atrial tachycardia. The origin of the arrhythmia was mapped to the secondary branches of the left lower pulmonary vein using an octapolar micro-mapping catheter. Immediately following application of three radiofrequency lesions, angiography of the left lower pulmonary vein revealed a region of focal stenosis at the site of energy application, with delayed pulmonary venous emptying. Attempts to relieve any element of spasm using direct administration of nitroglycerin were unsuccessful. Three months later repeat catheterization revealed an unchanged region of tight anatomical stenosis. Balloon dilation of two stenotic areas resulted in dramatic relief of the obstruction and improved venous drainage. Recatheterization 6 months later revealed mild restenosis that was successfully redilated. Intracardiac ultrasound demonstrated focal constriction. Care should be exercised in attempting RF ablation in distal arborization sites of the pulmonary veins in children, because of the small caliber compared to adult subjects. Radiofrequency induced focal areas of stenosis may be amenable to balloon catheter dilation.

Supine low-frequency power of heart rate variability reflects baroreflex function, not cardiac sympathetic innervation.

Background Power spectral analysis of heart rate variability (HRV) has been used to indicate cardiac autonomic function. High-frequency power relates to respiratory sinus arrhythmia and therefore to parasympathetic cardiovagal tone; however, the relationship of low-frequency (LF) power to cardiac sympathetic innervation and function has been controversial. Alternatively, LF power might reflect baro reflexive modulation of autonomic outflows. Objective We studied normal volunteers and chronic autonomic failure syndrome patients with and without loss of cardiac noradrenergic nerves to examine the relationships of LF power with cardiac sympathetic innervation and baroreflex function. Methods We compared LF power of HRV in patients with cardiac sympathetic denervation, as indicated by low myocardial concentrations of 6-[18F]fluorodopamine-derived radioactivity or low rates of norepinephrine entry into coronary sinus plasma (cardiac norepinephrine spillover) to values in patients with intact innervation, at baseline, during infusion of yohimbine, which increases exocytotic norepinephrine release from sympathetic nerves, or during infusion of tyramine, which increases non-exocytotic release. Baroreflex-cardiovagal slope (BRS) was calculated from the cardiac interbeat interval and systolic pressure during the Valsalva maneuver. Results LF power was unrelated to myocardial 6-[18F]fluorodopamine-derived radioactivity or cardiac norepinephrine spillover. In contrast, the log of LF power correlated positively with the log of BRS (r = 0.72, P < 0.0001). Patients with a low BRS (⩽ 3 msec/mm Hg) had low LF power, regardless of cardiac innervation. Tyramine and yohimbine increased LF power in subjects with normal BRS but not in those with low BRS. BRS at baseline predicted LF responses to tyramine and yohimbine. Conclusion LF power reflects baroreflex function, not cardiac sympathetic innervation

Neurally mediated cardiac syncope: autonomic modulation after normal saline infusion

OBJECTIVES This study assessed the heart variability response to orthostatic stress during tilt table testing before and after normal saline administration. BACKGROUND The efficacy of sodium chloride and mineralocortoid in the treatment of neurally mediated cardiac syncope is attributed to intravascular volume expansion; however, their modulation of autonomic nervous system activity has not been evaluated. METHODS Heart rate variability analysis was performed on 12 adolescents with a history of syncope or presyncope (mean age 15.2+/-0.7 years) during tilt table testing. Subjects were upright 80 degrees for 30 min or until syncope. After normal saline administration, the patient was returned upright for 30 min. Heart rate variability analysis data were analyzed by an autoregression model (Burg method). RESULTS All subjects reproducibly developed syncope during control tilt table testing; median time to syncope was 9.4+/-2.1 min. After normal saline infusion, none of the subjects developed syncope after 30 min upright. In the control tilt, there was an initial increase followed by a progressive decrease in low frequency power until syncope. Repeat tilt after normal saline administration demonstrates that low frequency power increased but the magnitude of initial change was blunted when compared with control. In addition, low frequency power increased during normal saline tilt sequence compared with the control tilt, during which it decreased. CONCLUSIONS Normal saline blunted low frequency power stimulation and prevented paradoxical low frequency power (sympathetic) withdrawal. Increasing intravascular volume with normal saline alters autonomic responses that may trigger neurally mediated syncope reflexes.

Postoperative Junctional Ectopic Tachycardia: Risk Factors for Occurrence in the Modern Surgical Era

Postoperative (PO) junctional ectopic tachycardia (JET) can be a life‐threatening arrhythmia that follows surgical repair of congenital heart disease (CHD) and results in PO morbidity.

Simultaneous heart rate and blood pressure variability analysis: Insight into mechanisms underlying neurally mediated cardiac syncope in children

OBJECTIVES The purpose of our investigation was to examine serial changes in autonomic nervous system activity along with measurements of hemodynamics and cardiac contractility, in assessing the mechanism(s) that underlie neurally mediated cardiac syncope (NMCS) in children. BACKGROUND Previous research that used heart rate variability analysis alone to understand changes in autonomic activity that result in NMCS has provided conflicting results. We performed simultaneous heart rate and blood pressure variability analyses to characterize dynamic alterations in sympathetic and vagal tone during tilt-table testing in 23 children with a history of syncope or frequent dizziness. METHODS Power spectra of heart rate and blood pressure variability were analyzed using autoregressive modeling. Maximum dP/dT of systolic blood pressure and the electrical-mechanical activation time were used to assess cardiac contractility. RESULTS Tilt-table testing was positive in 12 children and negative in 11. Syncope was associated with decreased heart rate, blood pressure and low-frequency (LF) power. Before episodes of syncope, systolic blood pressure dP/dT decreased, and the electrical-mechanical activation time was prolonged. The decrease in blood pressure LF power exceeded and occurred before the decrease in heart rate LF power. Despite similar early increases in LF power to the initial stress of upright tilting, no significant decline in LF power (heart rate or blood pressure) was observed during negative tilt-table tests. CONCLUSIONS All of these changes considered in total provide evidence supporting the hypothesis of sympathetic withdrawal/failure, resulting in a decrease in peripheral vascular tone and cardiac contractility, which results in profound hypotension in children with NMCS.

Managed Ventricular Pacing in Pediatric Patients and Patients With Congenital Heart Disease

Ventricular dyssynchrony induced by ventricular pacing (VP) may predispose patients to congestive heart failure. The detrimental effects of VP are directly related to the cumulative percentage of VP (Cum%VP). Managed VP (MVP) is a novel pacing algorithm developed to minimize unnecessary VP by uncoupling atrial pacing from VP. This retrospective analysis assessed the feasibility of using MVP in pediatric patients and patients with congenital heart disease (CHD). A multicenter review evaluated all pediatric patients <22 years old and older patients with CHD that had an implanted device using a MVP algorithm. Primary outcome variables were Cum%VP and adverse events. A subgroup analysis evaluated patients that had a DDD(R) pacemaker before a MVP device and compared Cum%VP before and after initiation of MVP. From 6 centers 62 patients (mean age 21.5 +/- 9.6 years) were included; 64% had CHD. With a MVP device, mean Cum%VP was 4.3 +/- 14.6% (range 0 to 83.7): Eleven patients were eligible for subgroup analysis. Compared with DDD(R), Cum%VP significantly decreased with MVP (67.1 +/- 29.4% vs 9.2 +/- 24.8%, p = 0.002). One MVP-related adverse event occurred; a patient with intermittent atrioventricular block had symptoms with frequent nonconducted atrial depolarizations and was reprogrammed to DDD. In conclusion, MVP can be used safely and can significantly reduce unnecessary VP in pediatric patients and patients with CHD.

Inadvertent detection of 60-Hz alternating current by an implantable cardioverter defibrillator.

LEE, S.W., et al.: Inadvertent Detection of 60‐Hz Alternating Current by an Implantable Cardioverter De‐fibrillator. A patient with an ICD received therapies from his ICD while exercising in an indoor swimming pool. Interrogation of the ICD revealed inappropriate detection of 60‐Hz alternating current artifact and delivery of ICD therapies.