Background:
Data on atrial fibrillation (AF) ablation and outcomes are limited in patients with congenital heart disease (CHD). We aimed to investigate the characteristics of patients with CHD presenting for AF ablation and their outcomes.
Methods:
A multicenter, retrospective analysis was performed of patients with CHD undergoing AF ablation between 2004 and 2020 at 13 participating centers. The severity of CHD was classified using 2014 PACES/HRS guidelines. Clinical data were collected. One-year complete procedural success was defined as freedom from atrial tachycardia or AF in the absence of antiarrhythmic drugs or including previously failed antiarrhythmic drugs (partial success).
Results:
Of 240 patients, 127 (53.4%) had persistent AF, 62.5% were male, and mean age was 55.2±0.9 years. CHD complexity categories included 147 (61.3%) simple, 69 (28.8%) intermediate, and 25 (10.4%) severe. The most common CHD type was atrial septal defect (n=78). More complex CHD conditions included transposition of the great arteries (n=14), anomalous pulmonary veins (n=13), tetralogy of Fallot (n=8), cor triatriatum (n=7), single ventricle physiology (n=2), among others. The majority (71.3%) of patients had trialed at least one antiarrhythmic drug. Forty-six patients (22.1%) had reduced systemic ventricular ejection fraction <50%, and mean left atrial diameter was 44.1±0.7 mm. Pulmonary vein isolation was performed in 227 patients (94.6%); additional ablation included left atrial linear ablations (25.4%), complex fractionated atrial electrogram (19.2%), and cavotricuspid isthmus ablation (40.8%). One-year complete and partial success rates were 45.0% and 20.5%, respectively, with no significant difference in the rate of complete success between complexity groups. Overall, 38 patients (15.8%) required more than one ablation procedure. There were 3 (1.3%) major and 13 (5.4%) minor procedural complications.
Conclusions:
AF ablation in CHD was safe and resulted in AF control in a majority of patients, regardless of complexity. Future work should address the most appropriate ablation targets in this challenging population.
Introduction:
Myxomas are the most common primary cardiac tumor, with clinical manifestations depending on tumor size and location. We present a case of giant left atrial (LA) myxoma causing functional mitral stenosis and associated severe pulmonary hypertension.
Case:
A 75-year-old woman with newly diagnosed heart failure and atrial fibrillation presented to the emergency department with 2-months of chest pain and shortness of breath. She had left-sided chest pain during exertion and at rest, dyspnea, and 3-pillow orthopnea. She was normothermic, blood pressure of 93/57 mm Hg, HR 63 bpm, respiratory rate of 21, and 91% oxygen saturation on room air. Cardiovascular exam revealed no murmurs, elevated JVD and bilateral lower extremity edema. Chest CT revealed a large mass, measuring 9x4 cm, in the LA extending into the left ventricle. TTE demonstrated a large mobile mass in the LA measuring 9.4x3.9 cm. The mass extended through the mitral valve into the left ventricle, causing functional mitral stenosis with mean inflow gradient of 10 mmHg. Severe tricuspid regurgitation was demonstrated with a peak regurgitant velocity of 4.89 m/s, with estimated right ventricular systolic pressure of 101 mmHg. Pre-operative pulmonary artery systolic pressure was 100 mmHg. She underwent surgical excision. Histology confirmed diagnosis of myxoma, and the mass measured 7.2 x 5.0 x 4.7 cm. At follow up, she had no heart failure symptoms or additional episodes of atrial fibrillation. Repeat TTE demonstrated normal RVSP.
Discussion:
Symptoms of myxoma include cardiovascular, embolic and constitutional symptoms. While obstructive symptoms are common in large myxomas, only 10% have severe functional mitral stenosis. Despite this, sequela of pulmonary hypertension and tricuspid regurgitation is not well documented. Diagnosis is made by TTE, which also allows assessment of valvular and hemodynamic changes. Treatment requires surgical resection, with low rates of recurrence.
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