Noncompaction cardiomyopathy and multiple coronary-cameral fistulae in an octogenarian
1Harborview Medical Center, University of Washington School of Medicine, 325 9 Avenue, Box 359748, Seattle, WA 98104, USA.
Insights
This case study highlights a rare instance of a patient with both noncompaction cardiomyopathy and coronary cameral fistulae. Management focused on heart failure care and medications for chest pain, with positive outcomes.
Area of Science:
- Cardiology
- Developmental Biology
Background:
- Noncompaction cardiomyopathy and coronary cameral fistulae are rare cardiac conditions.
- Their co-occurrence in a single patient is exceptionally uncommon.
Observation:
- An 85-year-old male presented with chest pain, diagnosed with left ventricular hypertrophy, and strain pattern on ECG.
- Echocardiography revealed regional noncompaction and hypokinesis.
- Coronary angiography identified diffuse coronary cameral fistulae involving all three coronary arteries.
Findings:
- The patient was diagnosed with coexisting noncompaction cardiomyopathy and coronary cameral fistulae.
- Medical management included beta-blockers and ACE inhibitors.
- Intervention for fistulae was not feasible due to their diffuse nature.
Implications:
- This case suggests a potential link between noncompaction cardiomyopathy and coronary cameral fistulae, possibly arising from a developmental anomaly.
- Effective management involves standard heart failure care and addressing potential coronary ischemia.
- The patient demonstrated a favorable outcome with medical management alone.
Abstract:
An 85-year-old man was admitted to the emergency department with chest pain. His electrocardiogram showed a right bundle branch block as well as increased voltages suggesting left ventricular hypertrophy and t-wave inversions consistent with a strain pattern (versus ischemia). He underwent echocardiography which showed regional noncompaction and associated hypokinesis. These findings led to coronary angiography which revealed multiple coronary-cameral fistulae involving all three coronary arteries. He was initially treated for acute coronary syndrome but after his diagnostic procedures this was narrowed to a beta blocker, to reduce myocardial oxygen demand, and an angiotensin-converting enzyme inhibitor due to the cardiomyopathy. Although the fistulae may have caused the patient's chest pain, intervention was not possible due to the diffuse nature of the fistulae. He did well in follow-up without the development of heart failure symptoms or continued angina. <Learning objective: Noncompaction cardiomyopathy and coronary cameral fistulae are two rare disorders that have even more rarely been described in a single patient. They may be a part of a spectrum of a single disease that results from arrest of the normal sequence of embryologic development of the heart. The management of the two conditions includes aspects of standard heart failure care as well as medical and possibly interventional therapy for coronary ischemia (angina) related to fistulae.>.
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