From the Guidelines
Yamaguchi syndrome and coronary fistula require distinct management approaches, with a focus on reducing symptoms and preventing complications, as outlined in the guidelines from the American Heart Association 1.
Management of Yamaguchi Syndrome
Yamaguchi syndrome, also known as apical hypertrophic cardiomyopathy, is typically managed with beta-blockers (such as metoprolol 25-100 mg twice daily) or calcium channel blockers (like verapamil 120-480 mg daily in divided doses) to reduce heart rate, improve diastolic filling, and alleviate symptoms.
- Comprehensive cardiac evaluation including echocardiography, cardiac MRI, and sometimes coronary angiography is necessary for accurate diagnosis.
- Patients with Yamaguchi syndrome should undergo regular cardiac follow-up, avoid dehydration, and consider genetic counseling, as it may have a hereditary component.
- Anticoagulation may be necessary in some cases of Yamaguchi syndrome if atrial fibrillation develops.
Management of Coronary Fistula
A coronary fistula is an abnormal connection between a coronary artery and another cardiovascular structure, which may require closure if symptomatic or hemodynamically significant.
- Small, asymptomatic fistulas can be monitored with regular cardiac evaluations, while larger ones causing symptoms or complications may need transcatheter closure or surgical repair.
- Embolization can be performed with coils or other occluding devices, but complications may include incomplete occlusion with residual shunting, myocardial ischemia, and distal embolization of a coil to the right side of the heart or the pulmonary artery, which requires retrieval, as noted in the guidelines 1.
- Periodic clinical evaluation with imaging such as echocardiography to assess both the size of the fistula and ventricular function is reasonable, as increasing fistula and shunt size may be associated with increased abnormalities of coronary flow and complications, including chest pain, decreased life expectancy, and risk of rupture 1.
From the Research
Yamaguchi Syndrome and Coronary Fistula
- Yamaguchi syndrome, also known as apical hypertrophic cardiomyopathy, is a rare variant of cardiomyopathy that affects the apical region of the left ventricle 2, 3, 4.
- It is frequently misdiagnosed or missed because its symptoms are extremely similar to those of acute coronary syndrome, including chest pain, palpitations, shortness of breath, and syncope 2, 3, 4.
- Coronary artery fistulas (CAFs) are rare coronary anomalies that are usually diagnosed incidentally with cardiac imaging, and can also cause symptoms similar to coronary syndrome, such as myocardial ischemia 5, 6.
- While there is no direct evidence linking Yamaguchi syndrome and coronary fistula, both conditions can present with similar symptoms and require careful diagnosis and management.
- Transcatheter closure of coronary artery fistulas has been shown to be a effective treatment option, with a favorable acute procedural success and complication rate in selected patients 5, 6.
- Early recognition and accurate diagnosis of both Yamaguchi syndrome and coronary fistula are crucial for better outcomes, and clinicians should consider these conditions in the differential diagnosis of chest pain and other cardiovascular symptoms 2, 3, 4.