A Case With Apical Hypertrophic Cardiomyopathy, Multiple Coronary Artery–Left Ventricular Fistulae, and a Morphological Structure Mimicking Left Ventricular Noncompaction
Statue of Cerberus or Double-Headed Eagle?
Multiple coronary fistulae that drain into the left ventricle are rarely associated with apical hypertrophic cardiomyopathy.1 On the other hand, associations of left ventricular noncompaction (LVNC) and multiple coronary fistulae or hypertrophic cardiomyopathy have previously been reported in several cases.2,3 We report here a unique case of apical hypertrophy of the left ventricle, multiple coronary–left ventricular fistulae, and a morphological structure of the left ventricular myocardium mimicking LVNC.
A 59-year-old man was admitted to our hospital for fever and rapidly progressing dyspnea that had persisted for 10 days. He had been diagnosed with apical hypertrophic cardiomyopathy in his 30s, although he had no regular medical checkups and was asymptomatic. On admission, the patient had mild wheezes audible in the midportion of the right lung and a diastolic murmur heard at the Erb and the cardiac apical area.
Chest x-ray and computed tomography revealed cardiac enlargement with increased pulmonary vasculature, granular shadows in the lower areas of both lungs, and increased density of the right middle lung area. These findings suggested failure of the left side of the heart with pneumonia. A 12-lead electrocardiogram (ECG) confirmed normal sinus rhythm with inverted T waves in leads II, III, aVF, and V3 through V6 (Figure 1). He recovered rapidly with antibiotics and standard therapy for heart failure.
Transthoracic 2-dimensional echocardiography showed diffuse hypertrophy of the mid to apical left ventricular walls (Figure 2A and Movie I in the online-only Data Supplement). Doppler echocardiography revealed multiple areas of mosaic color flow within the anterior, lateral, and apical myocardium connected to the left ventricular cavity, with a peak flow speed of 0.8 to 0.9 m/s in the diastolic phase (Figure 2B and Movie II in the online-only Data Supplement). Enlarged left and right coronary arteries and rapid diastolic flow with a peak speed of 1.5 m/s in the left anterior descending artery were also identified.
A markedly enlarged, tortuous coronary artery with multiple coronary artery–left ventricular fistulae was visible on cardiac computed tomography examination (Figure 3A). Coronary computed tomography angiography, diastolic-phase imaging, showed a 2-layer left ventricular myocardium mimicking LVNC, with a maximal ratio of noncompacted to compacted myocardium of 2.2 (Figure 3B and 3C).
Coronary angiography confirmed multiple fistulae between the left and right coronary arteries and the left ventricle (Figure 4A and 4B). Left ventricular angiography revealed a spade-shaped cavity indicative of apical hypertrophic cardiomyopathy.
Cine magnetic resonance images showed small fine trabeculae extending from the lower base to the apical anterior, lateral, and inferior endocardium in the diastolic phase. In the systolic phase, thickening of both layers of the myocardium (mimicking compacted and noncompacted myocardium) was present (Movies III and IV in the online-only Data Supplement).
Coronary artery fistula has been reported in ≈0.2% of patients undergoing coronary angiography, and other cardiac abnormalities are associated in 20% of those cases. Coexistence of multiple coronary artery–left ventricular fistulae and apical hypertrophic cardiomyopathy is very rare, and most drain from the right and left coronary arteries to the left ventricle.1 This phenotype is considered to represent the partial persistent embryonic myocardial sinusoids that arise from the endothelial protrusion into the intertrabecular spaces.1
The association of multiple coronary artery–left ventricular fistulae and LVNC was reported by Dias et al,2 with persistent myocardial sinusoids and compaction of the myocardium present in the same patient.
LVNC has gained increasing recognition in the last 30 years, and there has been some discussion of its terminology and definition.4 Our case fulfilled the morphological definition of LVNC, although there may be some suspicion that this structure really represents an arrest of the myocardial compaction process during the first trimester. Oechslin and Jenni4 described that multiple persistent sinusoids are generally present with pulmonary artery atresia and that these patients should not be included the category of LVNC. Unlike the Oechslin and Jenni report, the patient described here did not have pulmonary atresia. Coexistence of hypertrophic cardiomyopathy and LVNC in one family with overlapping phenotypes has been reported.3 A comprehensive diagnostic assessment, including multimodality imaging, systematic screening, and genetic assessment, may help elucidate the true cause of cases with multiple anomalies.
To the best of our knowledge, this is the first report describing a patient with apical hypertrophic cardiomyopathy, multiple coronary artery–left ventricular fistulas, and a morphological structure mimicking LVNC.
We thank Dr Naoto Fukuda for the computed tomography image acquisition and Professor Fukiko Ichida, Dr Takaaki Matsuyama, and Professor Katsu Takenaka for their kind academic suggestions.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.114.015008/-/DC1.
- © 2015 American Heart Association, Inc.
- Yuan L,
- Xie M,
- Cheng TO,
- Wang X,
- Zhu F,
- Kong X,
- Ghoorah D.
- Oechslin E,
- Jenni R.