Case Presentation by Dr. Kristi Maso
8 week old male presents to the ED with his mother who states the child has been irritable while feeding for 3 weeks, but worse today. She states she took him to his PCP 3 weeks ago and was diagnosed with acid reflux, however his irritability and sweating while feeding continued despite being started on zantac. Today when feeding the child turned blue in the face and according to mom seemed to “start breathing hard and really fast.” She became nervous and brought him to the ED. The child has been otherwise healthy. He is having normal bowel movements and wet diapers. No fever, vomiting, or diarrhea.
FH: No HTN, DM, or asthma
Birth Hx: Patient was full term, born at 39 weeks via normal spontaneous vaginal delivery
SH: Child lives at home with mom and dad. There is no smoking in the home and he does not go to daycare.
BP: not attainable child is crying, HR: 170, RR: 45, T: 36.7, Spo2: 90% on room air
Constitutional: Child is crying and irritable, he has a blue tint to his face.
Eyes: PERRL, EOMI, conjunctiva are pink without pallor
Cardiovascular: Tachycardic rate and regular rhythm. + holosystolic murmur, no rubs or gallops. No S3 appreciated. Good palpable radial pulses bilaterally.
Respiratory: + accessory muscle use. Crackles at the lung bases bilaterally. Faint expiratory wheezing.
Gastrointestinal: Abdomen is soft, non tender, nondistended. The liver is palpable 1cm below the costal margin.
Skin: Ashen color, no rash
Labs/imaging: A chest xray is obtained in the ED which showed mild cardiomegaly with mild pulmonary vascular congestion. Because of this an EKG was ordered .
All laboratory studies are still pending at this time.
1) Based on the patient’s history and EKG findings, which of the following is the most likely diagnosis?
a) Viral Myocarditis
b) Congenital Dilated Cardiomyopathy
c) Mitral Valve Prolapse
d) Anomolous Left Coronary Artery from Pulmonary Artery
e) Total Anomolous Pulmonary Venous Return
2) Which of the following is the treatment of choice for this child when presenting to the ED?
a) Place patient on 100% O2
b) IV Lasix
c) Bolus 20cc/kg to increase venous return
d) Blood cultures and broad spectrum IV antibiotics
e) Breathing treatments of albuterol and ipratropium x3
3) Which of the following sign or symptom is least likely to be found on exam for this condition?
a) Heart murmur
b) Enlarged cardiac silhouette
d) Poor feeding
Discussion: This EKG is diagnostic of a congenital anomaly called Anomolous Left Coronary Artery from Pulmonary Artery (ALCAPA). ALCAPA is a serious congenital anomaly that occurs in about 1 in 300,000 live births. In normal anatomical development the left and right coronary arteries come off the aorta, however in children with ALCAPA during development the left coronary instead comes off the pulmonary artery resulting in left ventricular dysfunction secondary to ischemia once the pulmonary pressures decrease as expected after birth. Clinically, irritability increases as myocardial oxygen consumption increases (usually at times of feeding or crying). States of increased oxygen demand lead to infarction of the anterolateral left ventricular free wall which causes mitral valve papillary muscle dysfunction and variable degrees of mitral insufficiency.
Facts about ALCAPA:
- Usually diagnosed within the first 2 months of life – when pulmonary artery resistance drops to adult level. (in fetal development the pulmonary artery pressure equals that of the systemic circulation so fetus gets adequate perfusion of the myocardium from the pulmonary artery through the anomalous coronary, BUT after birth does the pulmonary artery contains only desaturated blood at low pressure which isn’t enough to perfuse the left ventricle especially at times of high demand like feeding or crying)
- As pulmonary resistance decreases the left coronary artery blood blow travels back into the pulmonary artery essentially “stealing” blood from the ventricle causing worsening ischemia and infarction of the anterolateral free wall of the left ventricle.
- Presents in a way similar to common pediatric conditions such as infantile colic, food intolerance, gastroesophageal reflux, and bronchiolitis.
- Patients present in symptoms of heart failure and evidence of myocardial ischemia on EKG is pathognomic for the condition. Cardiomegaly can be seen on chest xray.
Electrocardiogram almost always shows evidence of anterolateral myocardial infarction in the symptomatic patients. Inverted T waves can be seen in leads I and aVL. The left ventricular surface leads (V 5 -V 6 ) may also show deep Q waves and exhibit elevated ST segments and inverted T waves. EKG is pathognomonic for ALCAPA and cannot be missed by ED physicians.
- Abnormal heart rhythm
- Enlarged heart
- Murmur (holosystolic secondary to mitral regurg) – RARE
- Blue or purple tint to gums, tongue, skin and nails (cyanosis)
- Poor eating and poor weight gain
- Crying with feeds
- Rapid breathing or shortness of breath
- Profuse sweating, especially with feeding
- More sleepiness than normal
- Unresponsiveness (the baby seems “out of it” at times)
Initial management is supportive but temporary. This includes diuretics, afterload agent, inotropes as needed, similar to treatment of CHF in adults. The DIFFERENCE is the use of oxygen. Using 100% O2 may further reduce pulmonary vascular resistance and worsen coronary steal from the right coronary artery into the pulmonary arteries. Oxygen saturations in the mid 80’s is permissible in these patients.
Lasix: Given IV is 1mg/kg.
Definitive treatment is surgery which entails cutting the LCA from the pulmonary artery and trying to reattach to aorta or anastomose with subclavian.