Case Report
A full-term 16 day old and 3,5 kg weight newborn boy was admitted our
department with absence of femoral pulses and suspected coarctation of
the aorta. Echocardiography(ECHO) revealed an arcus aorta hypoplasia
with important aortic coarctation (35- 40 mm hg gradient with diastolic
extension) and the diameter of proximal transvers arch was 4,9 mm
(z-score -3,27), the diameter of distal transvers arch was 4 mm (z score
-2,62), and the diameter of isthmic arch was 2,8 mm (z-score -4,29). She
also had restrictive muscular outlet ventricular septal defect with no
evidence of left ventricular volume overload, bicuspid aortic
valve with mild stenosis, left aortic arch, systemic pulmonary
hypertension. There was no abnormality in the patient’s preoperative
laboratory values, and the neonate did not have a fever or cough
preoperatively.
Surgical repair was performed with median sternotomy and cardiopulmonary
bypass (CPB) was performed. During the arch repair, the antegrade
cerebral perfusion was initiated (ASCP) and the del Nido cardioplegia
was given. Aortic arch was reconstructed with anterior patch
augmentation technique and VSD was closed with pericardial patch via
right atriotomy. After the cessation of CPB, modified ultrafiltration
was performed and the skin was closed with a patch, leaving the sternum
open. The patient was transferred to the intensive care unit with
moderate doses of inotropic supports, and in stable hemodinamic
condition. The CPB, the aortic cross-clamping time, and the ASCP time
were 152 min ,75 min, and 35 min respectively. In the control ECHO
examination, no significant gradient was observed on arkus, and no
residual VSD was detected. Peritoneal dialysis was started in the
postoperative period. Sternum was closed on the postoperative day 2
uneventfully. Laboratory tests included normal white blood cell (WBC)
count (6.2 ku/ul, ref range <= 4 ku/ul) with elevated 77,9%
polymorphonuclear neutrophils, with low lymphocyte count of 750
cells/mm3 (ref range <=800 cells/mm3) on the postoperative day
2. The lymphocyte count (570 cells/mm3) was lower than the previous day,
and C-reactive protein (CRP) levels (7.7 mg / dL, ref interval
<= 0.5 mg / dL) were higher on the postoperative day 3.
Acute respiratory deteriation was occurred with refractory hypoxemia and
worsening hypercapnia despite lung protective ventilation and high
positive end-expiratory presssure (PEEP) theraphy on the postoperative
day 3 (Figure 1). Extracorporeal membrane oxygenation (ECMO) support was
used because of acute respiratory dysfunction on the postoperative day
4. Venoarterial ECMO support was initiated via 14 Fr venous cannula into
the right atrium and 8 Fr arterial cannula into the ascending aorta.
ECMO support was started at flow rate of 100 ml/kg/min (80-140
ml/kg/min) and arterial waveform demonstrated normal pulsatility. WBC
count decreased to 4 ku/ul with elevated 72% polymorphonuclear
neutrophils, and with low lymphocyte count of 690 cells/mm3. The
patient’s reverse transcription-polymerase chain reaction (RT-PCR)
testing for SARS-CoV-2 was positive with the sample taken from the
endotracheal tube on the postoperative day 4. Hydroxychloroquine and
favipiravir theraphy was started immediately and given during 7 days.
Heparin infusion was started and activated clotting time was maintained
with in the goal range of 180-220. We did not see major complications as
thrombosis or bleeding during the ECMO support. The initial
echocardiogram on ECMO showed normal LV systolic function. The patient
weaned from ECMO after 6 days support. Since oxygen saturation decreased
and respiratory functions deteriorated 7 days after leaving ECMO, the
ECMO support was applied again. After 18 days of care including
hydroxychloroquine and favipiravir theraphy RT-PCR testing for
SARS-CoV-2 was changed negative; however, there was no significant
improvement in the patient’s clinical condition. During these periods,
the patient’s sternum being open also made the patient vulnerable to
infections in addition to Covid 19. Candida parapsilosis
mediastinitis was occurred, and Sterotrophomonas maltophilia reproduced
in blood culture during second ECMO support. Unfortunately, the patient
could not survive and died on ECMO due to sepsis and multiorgan failure
despite extended antibiotic theraphy on the postoperative day 28.