Multivessel Coronary Artery Disease in a Patient With Noncompaction Cardiomyopathy With Coronary Artery-to-Left Ventricle Fistulas.

Coexistence of coronary artery-to-left ventricle fistula (CALVF) and noncompaction cardiomyopathy (NCC) is rare in patients with severe multiple coronary stenosis. We report CALVF in a 70-year-old man with acute coronary syndrome (ACS) and NCC. Left ventricular ejection fraction may not improve by total revascularization in this condition. (Level of Difficulty: Advanced.).

History of Presentation

A 70-year-old man presented to the emergency department with dyspnea and typical angina. He was afebrile, with blood pressure of 110/70 mm Hg, heart rate of 72 beats/min, and respiratory rate of 18 breaths/min. Cardiac examination showed regular heart rate without any murmurs. Chest x-ray revealed an enlarged cardiac silhouette (Figure 1). Electrocardiography showed sinus rhythm, left cardiac axis, first-degree atrioventricular block, ST-segment elevation in V1 to V3, and T-wave inversion in leads DI and aVL (Figure 2). Troponin T and B-type natriuretic peptide levels were elevated (49 ng/mL and 1,493 pg/mL, respectively). The initial diagnosis was acute coronary syndrome (ACS).

Figure 1

Chest X-Ray

An enlarged cardiac silhouette.

Figure 2

Electrocardiography

Sinus rhythm, left cardiac axis, first-degree atrioventricular block, ST-segment elevation in V1 to V3, and T-wave inversion in leads DI and aVL are shown.

Learning Objectives

To be familiar with the complications associated with PCI in patients with NCC.

To explore the potential coronary artery-to-left ventricle fistulas after PCI.

Past Medical History

He had a medical history of type 2 diabetes mellitus, hypertension, and dyslipidemia and 2 episodes of syncope in the past 2 years. He had no family history of inherited disorders, noncompaction cardiomyopathy (NCC), or coronary artery-to-left ventricle fistula (CALVF).

Differential Diagnosis

The differential diagnosis for this disorder included dilated cardiomyopathy, hypertensive cardiomyopathy, chronic coronary artery diseases, myocarditis, and Takotsubo syndrome.

Investigations

Because non–ST-segment elevation myocardial infarction was found, the patient underwent urgent coronary angiography. Diagnostic angiograms revealed multiple coronary lesions including subtotal occlusion of the mid right coronary artery (RCA) (Figure 3, Video 1), chronic total occlusion (CTO) of the mid left anterior descending coronary artery (LAD) (Figure 4, Video 2), and moderate stenosis of the ostial circumflex artery (left circumflex [LCX]) (Figure 5, Video 3). After consultation with the heart care team, a decision was made to revascularize the mid RCA as it was considered as the culprit lesion. Ticagrelor 180 mg was administered orally. A 4.0 × 30-mm drug-eluting stent (Xience, Abbott) was successfully deployed. We initially observed several large and small fistulas between the distal branches of the RCA and left ventricle (LV) (Figure 6, Video 4). He was transferred to the department of cardiology for follow-up and further clinical management. On the next day, a transthoracic echocardiogram showed severely impaired LV ejection fraction (LVEF) of 31%, apical hypokinesis, and elongated trabeculations in the LV apex (Figure 7, Video 5). NCC was confirmed based on echocardiographic and cardiac magnetic resonance findings, which revealed a noncompact/compact layer ratio of 2.4:1 (Figure 8A) and an apical trabeculation (Figure 8B). He still had chest pain that persisted despite optimal medical treatment. Therefore, he was shifted again to the catheterization laboratory for percutaneous coronary intervention (PCI) of CTO of mid LAD. The procedure was performed successfully using the antegrade wire escalation technique. A 3.0 × 38-mm drug-eluting stent (Orsiro, Biotronik) was placed with noncompliant balloon optimization. After the procedure, we observed a similar phenomenon of several small fistulas originating from the LAD draining into the LV cavity (Videos 6 and 7). LV ventriculography showed low LVEF and LV global hypokinesia (Video 8).

Figure 3

Subtotal Occlusion of RCA

RCA = right coronary artery.

Figure 4

Mid LAD CTO

CTO = chronic total occlusion; LAD = left artery descending artery; LCX = left circumflex; LM = left main; OM = obtuse marginal.

Figure 5

Stenosis of the Ostium of LCX

Abbreviations as in Figure 4.

Figure 6

Coronary Artery-to-Left Ventricle Fistulas

(A) Small fistulas flow after RCA stenting. (B) Large fistulas flow after RCA stenting. LV = left ventricle; PL = posterior lateral; PDA = posterior descending artery; other abbreviation as in Figure 1.

Figure 7

Transthoracic Echocardiogram

Severely impaired LV contractility, apical hypokinesis, and elongated trabeculations are shown. Abbreviation as in Figure 6.

Figure 8

Cardiac Magnetic Resonance Findings

(A) Cardiac magnetic resonance findings show noncompact/compact layers ratio 2.4/1. (B) Apical trabeculation.

Management

He was prescribed aspirin, ticagrelor, statin, bisoprolol, furosemide, spironolactone, dapagliflozin, and valsartan/sacubitril. After 3 months, he was referred to our center again for cardiac evaluation after an episode of chest pain and dyspnea. Because of persistent chest pain, elective angiography was performed, revealing that the stent was patent and the fistula shunts between coronary arteries and LV remained similar. It was decided not to perform further PCI to the LCX artery as it may cause more coronary-to-ventricular shunting.

Discussion

To the best of our knowledge, this is the first report on CALVFs after PCI in a patient with NCC. NCC is a rare disorder with a very low prevalence of 0.05% to 0.14% among adults referred for echocardiographic examination.1, 2, 3 The simultaneous presence of NCC and CALVF is even less often reported in the medical literature. Our patient was hospitalized with suspicion of ACS, and PCI was performed followed by drug-eluting stent placement to the culprit RCA stenosis as appropriate. After the first angioplasty, there were several CALVFs draining blood from the RCA to the LV chamber, which may have caused “coronary steal syndrome.” Transthoracic echocardiography revealed a suspected NCC, and subsequent cardiac magnetic resonance findings confirmed this diagnosis. After the first procedure, he still had chest pain and LVEF was low. Therefore, we successfully opened the CTO of mid LAD. Similar to the first-time outcome, CALVF to the LV chamber was clearly observed.

Coronary artery fistula (CAF) and NCC share some common etiology that occurs during early embryogenesis. In normal development, during the 4th through 8th weeks of the embryonic period, the compression of loosely arranged trabeculations creates the ventricle wall, and the sinusoidal connection is then transformed into the coronary capillary system. NCC pathology includes the compaction failure of trabeculations, due to which the myocardium remains in the initial state, resulting in NCC.,

The CAF flow may drain into the vena cava, coronary sinus, pulmonary veins, or cardiac chambers. Rarely, as in the present case, it drains into the LV. Fistulas may originate from a distinct channel, such as a coronary artery or one of its branches, or less frequently, as in this case, from a complexity of several small vessels., The CAF draining from multiple coronary vessels into the LV suggested the abnormal persistence of myocardial sinusoids, which is similar to the etiology of the patient’s LV noncompaction myocardium presented during the embryonic period. Angina, a typical myocardial ischemia symptom, may be caused by “coronary steal.” Although optimal medical treatment and total revascularization in this specific situation alone stabilized the condition, they may not improve cardiac function. The patient was scheduled for implantable cardioverter-defibrillator implantation to prevent sudden cardiac arrest.

Follow-Up

The patient still had mild chest pain during exercise despite optimal medical therapy during follow-up. Transthoracic echocardiography showed a similar LVEF of 30%. Repeat angiography performed after 3 months showed that CALVFs were still present with similar severity.

Conclusions

Occurrence of CAF to LV chambers in NCC after PCI is rare and requires more attention during angiography. Total revascularization may not improve overall LVEF in this condition.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.