May Be Sign of Complex Underlying Problem
Kamlesh K. Jha, MBBS, MD, FAAP; Saranya Ravichandran, MD, PGY3; Jovina Benedito, BSN, RN; Pragya Jha
A 40-week, appropriate for gestational age male with Apgar score 9 and 9 at one and five minutes was born vaginally to a 26-year-old, G2P1 mother. His birth weight, length and head circumference 3620 grams, 50.8cm and 35cm, respectively. The mother’s blood type was A positive; HIV, HBsAg, RPR, GBS and urine toxicology were all negative. Mom received adequate antenatal care and had two antenatal ultrasounds at 21 and 29 weeks of gestation. Mom had no medical problems. Five minutes after birth a paucity of left-upper extremity movement, specifically abduction at the shoulder, was observed; flexion at the elbow and weak grasp reflex compared to the right was also observed. A chest X-ray revealed no broken bones. The baby received routine newborn medications, namely; Erythromycin eye ointment, 1mg IM of vitamin K injection and 10 micrograms of Hepatitis B vaccine, which was given intramuscularly. The baby stayed with the mother and tolerated feeding without any problem.
At twenty-four hours of age, the baby was screened for Critical Congenital Heart Disease (CCHD), and it was negative (pre-ductal 95% and post-ductal 97%). One hour after screening for CCHD, the registered nurse (RN) noted intermittent tachypnea and tachycardia with a missing beat on the monitor without having desaturation. The RN reported this new development to the neonatologist. On evaluation, the neonatologist noted the vitals were within normal range except for the intermittent tachypnea for which the neonatologist decided to monitor baby in the special care unit with continuous cardiorespiratory monitoring and continuous clinical examinations. The neonatologist ordered an electrocardiogram which was reported normal for age. CBC, CXR and a blood culture were done and the baby was started on IV Ampicillin and Gentamicin. The CXR reported by the radiologist was unremarkable. The neonatologist discussed the findings with the radiologist and spoke about the possibility of performing a fluoroscopy to observe diaphragmatic movement. Based on the chest X-ray (Figure 1), in which the position of both domes of the diaphragm was in a normal position, the radiologist came to the conclusion that doing fluoroscopy and exposing the baby to unnecessary radiation should be avoided.
The baby continued to tolerate feeding and remained hemodynamically stable. The neonate was evaluated by the rehabilitation physician and at discharge the mother was advised to schedule a follow-up appointment with Schwab Rehabilitation Center.
At 48 hours of age, a systolic heart murmur was heard and the on-call neonatologist decided that he would consider doing an echocardiogram if the baby deteriorated or did not improve. At 66 hours of life, a capillary blood gas was done in room air and was unremarkable. At 72 hours of life, (DoL4), the morning RN, before getting a sign-out from the night RN, observed the monitor beeping frequently. The morning RN saw the baby and thought the baby appeared mottled, breathing fast with retractions. The morning RN asked the night RN to call the neonatologist to evaluate. After the neonatologist evaluated the baby, he ordered HFNC 3.5L 30% FiO2 and decided to transfer the baby to the tertiary care center for further evaluation and management. The morning RN saw the baby saturating 99-100% and reduced FiO2 to 21% and flow to 2L. The morning RN said she did not want to close the PDA (Patent Ductus Arteriosus) by giving high oxygen. ABG at 74 hours of life, on 3L flow with 21% FiO2, was 7.34/37/45/20. Before the neonate was transferred, the morning RN repeated the screen for CCHD. It was a positive screen with pre-ductal saturation of 70% and post-ductal saturation of 100%. Later, the neonate was transported in the ambulance with HFNC.
On the way to the tertiary care center, the transport team, which had a nurse and respiratory therapist, along with EMS personnel, increased FiO2 to 100%. The baby did not deteriorate, but immediately on arrival to the baby suffered a hemodynamic collapse. Subsequently, the neonate was intubated and placed on SIMV pressure support ventilation. A Stat Echocardiogram was ordered. ABG was done which revealed metabolic acidosis with high PaO2 (7.16/19/192/6.8/-19.7). The EKG revealed Vent rate of 144bpm, atrial rate of 144bpm, P-R interval 110ms, QRS duration 086ms, QT interval 284ms, P-R-T axes 066-119-043 degrees, and QTc interval 439ms. Right atrial enlargement and right ventricular hypertrophy were observed. Absent q waves and prominent p waves suggested severe right-sided hypertrophy. An echocardiogram reported a hypoplastic left heart with moderately restricted ASD, restrictive PDA, all right-to-left with a gradient of 30-40mmhg and aortic and mitral valve atresia. Contractility of heart was satisfactory. A PGE infusion was started after the echo report and the patient was then transferred to the cardiac center where he underwent cardiac surgery.
To read the full article, please go to the February 2019 Issue of CCT.