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Coronary Anomalies: Recognition for Residents

Learning Objectives

By the end of this rotation, you should be able to:

  1. Recognize the classic ALCAPA presentation in infants
  2. Identify concerning symptoms that suggest coronary anomaly
  3. Know when to urgently refer to cardiology
  4. Understand why these are sudden death risks

Two Types to Know

ALCAPA (Anomalous Left Coronary Artery from Pulmonary Artery)

What it is: Left coronary artery comes from the pulmonary artery instead of the aorta.

When it presents: Infancy (as PVR drops, coronary steal occurs)

Classic presentation: - 2-3 month old with poor feeding, irritability, diaphoresis (anginal equivalent) - May appear to have colic or reflux - CHF symptoms: tachypnea, hepatomegaly - Echo shows dilated LV with poor function and MR

Key point: ALCAPA can mimic sepsis, myocarditis, or dilated cardiomyopathy. Think of it in any infant with unexplained LV dysfunction.

Treatment: Surgical emergency - coronary reimplantation

AAOCA (Anomalous Aortic Origin of Coronary Artery)

What it is: Coronary artery comes from the wrong sinus of Valsalva (e.g., left from right sinus).

Why it's dangerous: The anomalous vessel may travel between the aorta and pulmonary artery, where it can be compressed during exercise → ischemia → sudden death.

When it presents: Often asymptomatic until sudden cardiac death in an athlete.

Warning symptoms (if any): - Exertional chest pain - Exertional syncope - Exertional dyspnea

Red Flags - When to Refer

Urgent Referral

Finding Possible Concern
Infant with dilated cardiomyopathy Rule out ALCAPA
Infant with "colic" + diaphoresis + poor feeding Consider ALCAPA
Any child/teen with exertional chest pain + syncope Coronary anomaly, HCM
Athlete with chest pain during sports Must rule out AAOCA

Key Question to Ask

For any exertional symptom: "Do symptoms occur DURING exercise or after?" - During → concerning for cardiac cause - After → less concerning (but still evaluate)

What Happens After Referral

Cardiology will: - Obtain echocardiogram (may visualize coronary origins) - Consider CT angiography or MRI for AAOCA - Determine surgical need based on anatomy and symptoms - Restrict from sports pending workup

Key Teaching Points for Residents

  1. ALCAPA presents in infancy - think of it in any infant with DCM
  2. "Colic" with diaphoresis = consider angina in infants
  3. Exertional chest pain + syncope in athletes = urgent cardiology referral
  4. AAOCA can cause sudden death during exercise with no prior warning
  5. These are surgically correctable - early recognition saves lives

Key Guidelines

2017 AATS Guidelines for the Diagnosis and Management of Anomalous Aortic Origin of a Coronary Artery J Thorac Cardiovasc Surg. 2017;153(6):1440-1457 PMID: 28274557

Board Pearls

Pearl: ALCAPA = infant with dilated cardiomyopathy + mitral regurgitation

Think ALCAPA in any infant with unexplained LV dysfunction. Classic presentation is 2-3 month old with "colic," diaphoresis, and poor feeding (anginal equivalents).

Pearl: Exertional symptoms in athletes = rule out AAOCA before clearance

Chest pain, syncope, or dyspnea DURING exercise mandates cardiology evaluation. AAOCA can cause sudden death with no prior warning.

Pearl: ALCAPA is surgically curable - early recognition saves lives

Once diagnosed, surgical coronary reimplantation has excellent outcomes. LV function often recovers completely.