81. Case Report: Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) – Massachusetts General Hospital

CardioNerds (Amit Goyal & Karan Desai) join Massachusetts General Hospital cardiology fellows (Daniel Pipilas, Rachel Frank and Kemar Brown) on a luxurious sailboat for iced coffees and Modern Pastry delicacies! They discuss a rare case of Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA). Program director, Dr. Doreen DeFaria Yeh provides the E-CPR and a message for applicants.  Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summary - Case media - Case teaching - References

Episode graphic by Dr. Carine Hamo

CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza

Patient Summary

A women in her early 30s who's a Jehovah's witness presented with three days of intermittent chest pain. Past medical history included anxiety. Initial vitals and physical exam were unremarkable. Labs were notable for an elevated troponin T of 360 ng/L and a low TSH of 0.02 mIU/L with an elevated free T4 of 5.1 ng/dL. EKG demonstrated lateral and inferior ST depressions. TTE demonstrated a normal LVEF of 58% with a subtle anterolateral wall motion abnormality. Given her lack of conventional risk factors for CAD, resolution of her chest pain, and downtrending troponin, coronary CTA was obtained next which did not show any CAD but demonstrated an anomalous left main coronary artery (LMCA) arising from the main pulmonary artery with evidence of left to right shunting from the left main into the PA and extensive coronary and bronchial collateralization. The anterior wall hypokinesis was also seen on CT, consistent with ischemia due to myocardial steal phenomenon. Given the abnormal thyroid function tests, thyroid US was also obtained which showed patchy heterogeneity consistent with thyroiditis. Ultimately, the patient was diagnosed with ALCAPA and her chest pain was attributed to steal phenomenon due to hyperthyroidism and increased cardiac demand. She was treated with long-acting nitrates and beta-blocker with resolution of symptoms and was referred to cardiac surgery on discharge.  

After a multidisciplinary discussion involving the cardiac surgery team, patient underwent ligation of LMCA with SVG bypass to LAD. One month after operation, she developed palpitations and chest pain during exertion and was taken to the hospital. Labs showed an elevated hs-troponin T of 711 ng/L and she was treated for type 1 NSTEMI with aspirin, heparin drip, and statin. Repeat TTE demonstrated normal LVEF and lack of WMA. LHC showed occlusion of SVG graft and possible thrombus in LAD near the site of graft anastomosis. RCA was large and patent, providing adequate collaterals to the left coronary system. Ultimately, PCI was deferred and medical management was pursued because she had adequate collaterals from right coronary system. She was treated with DAPT, beta-blocker, and atorvastatin and has been doing well since.  

Case Media

ABCDEFClick to Enlarge

A. CXRB. ECG C. Follow up ECGD-F. Cardiac CT

TTE 1

TTE 2

Angiography 1

Angiography 2

Angiography 3

Angiography 4

Angiography 5

Episode Schematics & Teaching

The CardioNerds 5! – 5 major takeaways from the #CNCR case

How are the coronary arteries formed during embryology and how are anomalous coronary arteries formed?  During embryology, according to one theory, the coronary ostia and artery formation begins with ingrowth of a capillary plexus into the aortic sinuses. This complex process heavily depends on the proliferation and migration of cells that originate outside the heart at the sinus venosus and then differentiate into endothelial cells, vascular smooth muscle cells, and fibroblasts.