Abstract:

Objective: To study the clinical features of a girl with noncompaction cardiomyopathy complicated with multiple coronary artery-to-right ventricle fistula and review the literatures. Methods: Clinical symptoms and assistant examinations such as electrocardiogram, echocardiography and cardiac catheter of the case diagnosed as noncompaction cardiomyopathy complicated with multiple coronary artery-to-right ventricle fistula were reviewed. Two cases of noncompaction cardiomyopathy complicated with coronary artery to ventricle fistula and part of patients with cardiomyopathy complicated with coronary artery to ventricle fistula reported since 2003 were also reviewed in the study. Results: (1)A 6 month girl with poor appetite and night sweat was admitted to our hospital. Transthoracic echocardiography revealed a moderately impaired left ventricular contractility (ejection fraction 34% calculated according to Simpson's rule) and hypertrabeculation of the left ventricular latero-apical region with a maximal ratio of noncompacted to compacted myocardium as 2.5, supporting the diagnosis of noncompaction cardiomyopathy. The color Doppler and contrast echocardiogram showed an unusually evident diastolic flow within the compacted layer of the myocardium. The coronary angiography showed the right ventricle was opaque after left and right coronary injections, disclosing multiple coronary right ventricular fistulae. The patient accepted digoxin, furosemidum, spironolactone treatment and the clinical symptom was improved. （2）Retrospective review of the literatures showed two cases of noncompaction cardiomyopathy complicated with coronary artery to ventricle fistula. These patients were adults and showed symptoms as dyspnea and angina pectoris. Doctors made a definite diagnosis through the examination of echocardiography and coronary angiography. One case of cardiomyopathy complicated with coronary artery to ventricle fistula accepted implantable cardioverter defibrillatror because of syncope. Conclusion: Noncompaction cardiomyopathy associated with multiple coronary artery-to-right ventricle fistulas is one of the rare disease. The clinical symptoms mainly focus on congestive heart failure such as poor appetite and night sweat in infant. The echocardiography and coronary angiography is helpful to make a diagnosis. The medicine such as digitalis, diuretic and angiotensin converting enzyme inhibitors can be used to improve symptoms.