Robert Stewart, MD
Fellow, Pediatric Emergency Medicine
LeBonheur Children’s Hospital
Other Contributors
Mindy Longjohn, MD
Vijay Joshi, MD
The Case
A full term two-month-old female presents in respiratory distress. She has been fussy for two days, and has become increasingly worse with feeds. Review of systems is positive for tactile fever, nasal congestion and cough. Prenatal history was unremarkable.
Physical Exam
HR 186 BP 112/63 RR 36 T 39 C Sat 93% on RA
General In respiratory distress
HEENT sunken fontanelle, tacky mucous membranes and dry lips
Respiratory Labored with global retractions but clear to auscultation bilaterally
Cardiac tachycardia, no murmurs and regular rhythm. capillary refill is 3 seconds. Pulses are 2+ peripherals and centrally
Abdomen soft, no organomegaly
A venous blood gas showed the following:
pH 7.11 PCO2 52 Bicarbonate 17 L Base excess 13
Lactic Acid was 9 MMOL/L (reference range 0.7-3.3)
The team then obtained the following tests which helped establish the ultimate diagnosis.
What is the diagnosis?
A. Dilated Cardiomyopathy
B. Anomalous Left Coronary Artery from the Pulmonary Artery
C. Coarctation of the Aorta
D. Tetralogy of Fallot
E. Sepsis and Pneumonia
B. Anomalous Left Coronary Artery from the Pulmonary Artery
The patient’s EKG and imaging were diagnostic for ALCAPA, thew congenital heart disease diagnosis that is not to be confused with the mammal that looks like a llama. The EKG showed pathologic Q waves in lead I, AVL, V5, and V. There was ST Inversion in V6 and ST changes as well. Left ventricular hypertrophy with QRS >25mm on V6 is consistent with anomalous left coronary artery from the pulmonary artery (ALCAPA).
Annotated EKG – Courtesy Robert Stewart
The bedside ultrasound, annotated version linked below, shows an area of infarction leading to papillary muscle dysfunction, tricuspid regurgitation, poor dysfunction, and heart failure.
Annotated Ultrasound (opens in a separate window)
The following annotated ultrasound reveal more anatomic and functional abnormalities.
Annotated Ultrasound – Showing anatomic features of ALCAPA (Opens in separate window)
The final annotated ultrasound with doppler shows “steal syndrome,” the left coronary artery branching off of the pulmonary artery with reflux into the pulmonary artery.
Steal Syndrome (Opens in separate window)
Ultimately the patient continued to decline and the team decided to intubate. They prepared by placing defibrillator pads on the patient and had a CPR board in place. Doses of epinephrine were at the ready. Despite this preparation the patient became apneic and bradycardic after atropine and versed for RSI. CPR was started, an advanced airway was secured and after epinephrine ROSC was obtained.
A little while later the patient underwent successful reimplantation of left coronary artery to the ascending aorta with reconstruction of main pulmonary artery. She is being followed by cardiology outpatient and is doing well.
In summary, think of ALCAPA in patients that present with fussiness that is worse with feeds and evidence of heart failure. The key finding on EKG is the presence of pathologic Q waves representing ischemia/infarct, along with ST changes and LVH.
ALCAPA – Courtesy CHOP
References
Anomalous Left Coronary Artery From the Pulmonary Artery in Infants and Toddlers Misdiagnosed as Myocarditis. Aaviva Levitas, Hana Krymko, Viktoriya Ioffe, Eliyahu Zalzstein, Arnon Broides. Pediatr Emerg Care. 2016 Apr; 32(4): 232–234. doi: 10.1097/PEC.0000000000000511
Changes Electrocardiographic Patterns During Medical Treatment in a Patient With Anomalous Left Coronary Artery Originating From the Pulmonary Artery Dona Brekke, Curt G. DeGroff and Michael Schaffer Circulation. 2001;103:e85-e86, originally published April 24, 2001 https://doi.org/10.1161/01.CIR.103.16.e85
