Summer 2021
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Case of the Month
Chief complaint: Congestion for 3 days
By Allison Kasper MD
Children's National Health System
History
The patient is a 10 day old female who presents with the complaint of congestion and heavy breathing for 3 days. Her parents report that she has been breathing quickly and sounding congested, but there is no cough and they have not seen rhinorrhea nor seen a change with nasal suctioning. There has been no cyanosis. There has been no fever. She is exclusively breastfed and has been nursing well with no sweating with feeds. She is voiding and stooling normally. At their first primary care visit, she was noted to have labored breathing and was sent in for further evaluation.
Physical with vital signs
Rectal temperature: 37.1 C, Heart rate 159, Respiratory rate 90, Pulse Oximetry 97%
General: Alert. Appropriate for age. Crying and appears distressed.
Skin: Warm. Pink.
Head: Normocephalic, atraumatic. Anterior fontanelle open and soft.
Eye: Pupils are equal, round and reactive to light. Normal conjunctiva.
Ears, nose, mouth and throat: Tympanic membranes clear. Oral mucosa moist. No pharyngeal erythema or exudate.
Cardiovascular: Regular rate and rhythm. No murmur. Normal peripheral perfusion. Unable to palpate femoral pulses.
Respiratory: Lungs are clear to auscultation. Tachypneic with nasal flaring, intercostal and subcostal retractions.
Gastrointestinal: Nontender. Rounded abdomen, but soft. Liver 2 cm below costal margin.
Genitourinary: Normal genitalia for age.
Musculoskeletal: Normal ROM. normal strength. No tenderness. No swelling.
Neurological: No focal neurological deficit observed.
Urgent care workup
Four extremity blood pressures obtained: right arm: 99/47; right leg 76/40; left arm 105/61,
left leg 84/65. Preductal and postductal SpO2 97% and 98%, respectively. EKG showed right axis deviation and possible right ventricular hypertrophy. Chest X-ray was normal
Disposition
The patient was sent to the Emergency Department based on severity of increased work of breathing and ultimately to the cardiac intensive care unit.
Ultimate diagnosis
On echocardiogram, the patient was diagnosed with congenital heart disease including aortic arch hypoplasia, aortic coarctation and large posterior malaligned ventricular septal defect. She went to the operating room for repair including aortic arch repair with augmentation using femoral vein homograft patch, pericardial patch repair of her VSD and ligation and division of her patent ductus arteriosus.
Learning pearls
- Aortic coarctation that presents in the neonatal period occurs at the time of the closing of the ductus arteriosus, which begins after birth due to increased arterial blood oxygen levels and decreased prostaglandin E2 levels, and is usually complete by 2 to 3 weeks of age. Outflow obstruction across the narrowed aorta can no longer be compensated for as the ductus arteriosus closes.
- Aortic coarctation is often associated with multiple other congenital heart malformations. Some of these include bicuspid aortic valve, single ventricle anatomy, and VSD.
- Neonates with coarctation of the aorta can be asymptomatic and not be diagnosed until much later in life, but can present with heart failure and shock. Ductal dependent congenital heart disease is important to include in the differential diagnosis for neonates presenting in the early postnatal period.
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