A RARE PRESENTATION OF CONGENITAL HEART DEFECT

Dr Anakha V Ajay   Dr Jayasree  Dr Perraju  Dr AshwinDr Lakshmi Dr Smrithi(Dept of Neonatology)Dr  C Jayakumar(Dept pf Pediatrics), AIMS, Kochi
Twenty one day old female ba was born to a thirty five year old primi mother at 34+6 weeks of gestation with birth weight of 2.3kg via emergency LSCS (Indi-Leaking PV). Mother was on insulin  and treatment for Hypothyroidism
Antenatal scan showing  Bilateral Superior vaena canvas seen with persistent left SVC to coronary sinus 
Accessory renal artery noted on left side. Ba cried immediately after birth. 
APGAR score 8/9 at 1/5 min.
On examination, ba was tachypneic with subcostalretractions.
Other vitals stable.Cardiorespiratory examination was within normal limits. Hence ba was started on free flow oxygen and shifted to NICU.
On arrival ba was connected to CPAP with Fio2 21 % and PEEP 6. Xray showed features suggestive of respiratory distress syndrome type 2.. Ba was initially started on IV fluids. Feeds were started within 4 hours of life and gradually increased. Ba was also started on IV Ampicillin and Amikacin after taking blood culture. Fluconazole prophylaxis was given in view of central line. CPAP support was tapered to nasal cannula 3l/min on day 5 of life.
In view of antenatal scans showing bilateral SVC seen with persistent left SVC to coronary sinus, screening ECHO was done which showed PFO with left to right shunt, Left SVC , 3 mm PDA with left to right shunt. Ba was noted to have systolic murmur from day 6 of life. As PDA was hemodynamically stable medical management for the same was not attempted. Ba was continued on HFNC support. As ba had persistent desaturation respiratory support was changed to HFNC after which distress settled with no further worsening. Repeat chest X- ray done showed mild haziness of bilateral lung fields. Ba was active, with normal tone. 
Neurosonogram done in view of dysmorphismwas reported normal. USG KUB done showed mildly lobulated margins and slighlty malrotated left kidney. Renal functions done was normal hence nil intervention was advised. 
On day 11 of life ba had hyperkalemia hence started on insulin and calcium gluconate which was stopped as serum potassium normalised. Serum cortisol level was done in suspicion of CAH and was normal. On day 13 in view of increased distress with fall of saturation respiratory support escalated to bubble CPAP.Microarray was not due to financial constraints 
On day 20 ba had increased respiratory distress on CPAP ,sepsis screen showed elevated CRP 
After taking blood culture ba was started on Meropenam and Cefperazone sulbactum. CRP showed rising trend. Detailed echo done showing Isolated RPA from ascending aorta left to right lung, Both ventricle appear dilated, Isolated PAPVC of right PV to low SVC 
Bilateral SVC, LSVC to roofed CS, PFO Left to right. Large PDA continuing as descending aorta. In view of left to right shunt with congested lung fields ba was continued on furosomide intravenously in addition to oral Digoxin and spironolactore .
The same day ba had worsening of respiratory distress. Blood gas was monitored closely which was found to be in the acceptable range. Further ba developed poor spontaneous breathing efforts and fall of blood pressure with bradycardia and feeble peripheral pulses. Ba was intubated and ventilated. Ba developed severe metabolic acidosis. Repeat ECHO showed worsening of ventricular function. Ventilator mode was changed to HFOV. Ba developed asystole and resuscitation was given as per NRP for 30 mins. Ba had a rapid down hillcourse and succumbed to death.
 
 
Congenital heart defects(CHD) are structural problems arising from abnormal formation of the heart or major blood vessels. CHDs occur in about 1 out of 100 babies. Most heart defects cause abnormal blood flow through the heart, lack of formation or a fault in one of the pumping chambers or blocked blood flow in the heart or vessels. In most cases the cause is unknown, or can be genetic or hereditary. Risk factors include: Type 1 or 2 DM, Alcohol consumption, Smoking, Certain medications etc. Clinical presentation includes: respiratory distress, cyanosis, poor feeding and weight gain, abnormal heart rate or heart sounds. Types are: Obstructive defects, Septal defects and Cyanotic defects. Diagnosis for majority of cases is antenatal via fetal echocardiography. Other tests done postnataly are ECG, Cardiac MRI, Cardiac CT scan, Cardiac catheterisation. Management depend on child’s individual condition. Some simple defects may just need to be monitored. Some condition may be treated using medications, Cardiac catheterisation is also used for some. Other conditions will require surgery.
Prognosis: Due to advances in early diagnosis, testing, treatment and surgeries, most patients with these conditions are living into adulthood. Prognosis depends on severity of disease. More severe block indicates poorer prognosis.