Dronedarone for the Treatment of Atrial Arrhythmias in Adults With Congenital Heart Disease.

BACKGROUND: Atrial tachyarrhythmias are common and difficult to treat in adults with congenital heart disease. Dronedarone has proven effective in patients without congenital heart disease, but data are limited about its use in adults with congenital heart disease of moderate to great complexity. METHODS: A single-center, retrospective chart review of 21 adults with congenital heart disease of moderate to great complexity who were treated with dronedarone for atrial tachyarrhythmias was performed. RESULTS: The median (IQR) age at dronedarone initiation was 35 (27.5-39) years. Eleven patients (52%) were male. Ten patients (48%) had New York Heart Association class I disease, 10 (48%) had class II disease, and 1 (5%) had class III disease. Ejection fraction at initiation was greater than 55% in 11 patients (52%), 35% to 55% in 9 patients (43%), and less than 35% in 1 patient (5%). Prior treatments included ß-blockers (71%), sotalol (38%), amiodarone (24%), digoxin (24%), and catheter ablation (38%). Rhythm control was complete in 5 patients (24%), partial in 6 (29%), and inadequate in 10 (48%). Two patients (10%) experienced adverse events, including nausea in 1 (5%) and cardiac arrest in 1 (5%), which occurred 48 months after initiation of treatment. There were no deaths during the follow-up period. The median (IQR) follow-up time for patients with complete or partial rhythm control was 20 (1-54) months. CONCLUSION: Dronedarone can be effective for adult patients with congenital heart disease and atrial arrhythmias for whom more established therapies have failed, and with close monitoring it can be safely tolerated.

Heart Block in Unpalliated Complex Adult Congenital Heart Disease: Massive Collateral Burden Precludes Epicardial Pacemaker.

Complete heart block is a common complication for adults with congenital heart disease (CHD). Epicardial pacing is preferred in patients with septal shunting due to risk of thromboembolism. Anatomic changes in complex CHD may preclude surgical epicardial lead placement. Thromboembolism risk reduction in such patients requiring endocardial pacing remains questionable. (Level of Difficulty: Advanced.).

Improvement in Protein-Losing Enteropathy in a Patient With Fontan Circulation After Cardiac Rehabilitation and Prescriptive Exercise.

Fontan-associated protein-losing enteropathy is difficult to treat and associated with poor prognosis. Cardiac rehabilitation and exercise are thought to have beneficial effects for patients with Fontan circulation. We report the case of a young adult patient palliated to Fontan circulation, with a decade-long history of symptoms related to protein-losing enteropathy. At age 23 years, he appreciated an improvement in symptoms and laboratory values after cardiac rehabilitation and prescriptive exercise.

Pulmonary Embolism After Vaginal Delivery in a Fontan Patient.

The Fontan procedure was created to address the mixing of pulmonary and systemic venous return in patients with a single functional ventricle. The patient in this case with a Fontan repair experienced multiple pulmonary emboli 10 days post-partum. We outline management and recommendations when treating these patients. (Level of Difficulty: Beginner.).

Management of Heart Failure in Adult Congenital Heart Disease.

Heart failure (HF) in the adult with congenital heart disease (ACHD) is associated with high morbidity and mortality and has been implicated as the leading cause of death in this patient population. The diagnosis of HF in ACHD involves a combination of clinical suspicion from subjective patient history, anatomic imaging, functional diagnostic studies, and rhythm evaluation. Once diagnosed, the approach to management of HF in this population varies widely and by lesion. Unfortunately, there is a paucity of literature available delineating the optimal management of these patients, making clinical decision-making extremely challenging. In this review, we aim to summarize available evidence to help guide the diagnosis and management of HF in ACHD.

The effects of lifestyle changes on serum lipid levels in children in a real life setting.

OBJECTIVE: Studies have shown improvement in lipid levels after institution of lifestyle changes in children enrolled in closely monitored programs. There programs are difficult to mimic in real world clinics. We aim to determine if diet and exercise result in improvement in lipid levels in patients seen in a designated lipid clinic in a real life setting. DESIGN: Retrospective review of patients followed for dyslipidemia at the Texas Children's Hospital Lipid Clinic from May 1, 2012 to May 1, 2015. Patients included were seen more than once, had repeat lipid testing, and abnormal baseline lipid levels. Multivariate analysis using mixed models were performed to compare outcomes in patients who did and did not participate in lifestyle change. RESULTS: Of the 268 patients seen within the study period, 174 (56% male, 44% female) met inclusion criteria. Median age was 11 years. Compared to patients who did not make lifestyle changes: patients who made only diet changes demonstrated significant improvement in weight only (slope = -1.55, P-value = .014), and those who made only exercise changes demonstrated significant improvements in serum cholesterol (slope = -22.8, P-value = .017) and non-HDL cholesterol (slope = -28.7, P-value = < .01) levels. Patients who participated in both diet and exercise demonstrated significant improvement in weight (slope = -1.13, P-value = .011), diastolic blood pressure (slope = -1.82, P-value = < .01), and serum lipid levels: LDL (slope = -10.8, P-value = 0.017), HDL (slope = 1.52, P-value = .24), Triglycerides (slope = -0.11, P-value = .033) compared to those who did not make lifestyle changes. CONCLUSIONS: Outpatient management of dyslipidemia is difficult. Only patients who participated in both diet and exercise showed significant improvement in outcomes when compared to those who did not make lifestyle changes.

Intra-procedural continuous dialysis to facilitate interventional catheterization in pediatric patients with severe renal failure.

BACKGROUND: Interventional catheterization procedures may be needed for patients with severe renal failure who are dependent on dialysis. To avoid the risk of fluid overload and electrolyte derangement during complex procedures in this oliguric/anuric patient population, we performed intra-procedural dialysis, either continuous renal replacement therapy (CRRT) or continous cycling peritoneal dialysis (CCPD). METHODS: We performed a retrospective review of a cohort of pediatric patients, ages 0-18 years, with dialysis-dependent renal failure who received CRRT or CCPD during catheterization procedures from January 2013 to March 2016. RESULTS: Eight patients underwent a total of nine interventional catheterization procedures while receiving intra-procedural dialysis. Median age was 4.5 years (range 8 months to 17 years) and weight, 11.6 kg (11.2-62.6 kg). Six patients had end-stage renal disease (ESRD) and two patients had acute kidney injury (AKI), one due to hepatorenal syndrome and one due to multifactorial causes associated with congenital heart disease. The most common reason for catheterization was occlusive venous thrombosis requiring recanalization. CRRT was used during five cases and CCPD during four cases. Median procedure time was 337 min (95-651 min) and median contrast dose 4.2 mL kg-1 (1.2-8.2 mL kg-1 ). Euvolemia was maintained based on pre- and post-catheterizations weights, and no significant electrolyte abnormalities occurred based on lab monitoring during and post-procedure. CONCLUSIONS: Intra-procedural dialysis using CRRT or CCPD enables even small pediatric patients with severe renal failure to undergo long and complex interventional catheterizations by reducing the risk of fluid overload and electrolyte abnormalities. Collaboration between nephrology, cardiology, and dialysis teams is necessary for successful management of this challenging patient population.

A night with Venus, a lifetime with Mercury: a case of multiple intracranial aneurysms.

A 62-year-old African American woman presented to her primary care physician with binocular photopsias increasing in frequency over 1 year. Magnetic resonance angiography of the brain revealed bilateral 3-mm internal carotid artery aneurysms. She was referred to vascular medicine for further work-up. Her physical examination was unremarkable except for a right-sided femoral bruit. Specific testing for causes of multiple intracranial aneurysms was unremarkable except for positive rapid plasma reagin titers and fluorescent treponemal antibody absorption staining. She was referred to Infectious Diseases clinic where it was determined that the patient had a history of congenital syphilis that was not adequately treated until almost 20 years after birth. Few case reports illustrate the relationship between syphilis and intracranial aneurysms. The authors present this case to increase awareness of this uncommon presentation.