The etiologies of pulmonary hypertension(PH) are very diverse and include congenital heart disease(CHD), parenchymal lung diseases, pulmonary hypoplasia, congenital diaphragmatic hernia, sepsis, perinatal depression, and in utero medication exposure (selective serotonin reuptake inhibitor and nonsteroidal anti-inflammatory)[1]. Although intrathoracic disease and congenital heart defects are the most common etiologies, extrathoracic causes and non-cardiac causes must be considered[2]. Congenital hepatic hemangioma (CHH) is an example of a potentially fatal prognosis if not positively managed. Here, we report the case of a newborn who presented with tachypnea and tachycardia. It has been established that the newborn’s symptoms were due to PAH secondary to CHH.
Case presentation
A 23-year-old primigravida was referred to our hospital at 37 weeks of gestation because the B ultrasound showed that the fetal right atrium was enlarged, the lung-to-host ratio was 0.69, and the hepatic vein was widened. Maternal history was unremarkable, with the routine first- and second-trimester ultrasound scans reported as normal. At 37 + 2 weeks, an elective cesarean section was performed, delivering a female infant weighing 2750 g and with Apgar scores of 9 and 10 at 1 and 5 min, respectively. On the first postnatal day, the physical examination revealed tachypnea and tachycardia. The breath sounds were equal bilaterally. Cardiovascular examination revealed a grade 3/6 holosystolic murmur best heard at the left parasternal second intercostal border, with no thrill. The liver was tangible 3 cm below the right costal margin. No vascular malformations were noted over the skin and no splenomegaly was not observed.
A complete blood count revealed mild anemia. Evaluation of cardiac function revealed markedly elevated (33198 pg/mL) B-natriuretic peptide (BNP). Liver function tests were all within normal limits apart from decreased total protein (45.9 g/L, normal range: 49–71 g/L) and albumin (30.4 g/L, normal range: 35–50 g/L). There was no evidence of renal function dysfunction (creatinine: 63µmol/L; blood urea nitrogen: 6.5 mmol/L). Serum α-fetoprotein level (> 3000ng/ml) was above normal limits for the neonatal period. There was no evidence of hepatitis B or A virus infection.
We performed echocardiography, which revealed a heart with a maximum tricuspid regurgitation jet velocity of 4.7 m/s (estimated pulmonary artery systolic pressure of 88 mm Hg), atrial septal defect (ASD) 4.9 mm (bidirectional shunt), patent ductus arteriosus (PDA) 3.0 mm (bidirectional shunt), dilated right atrium and right ventricle, indicative of PH. Chest radiograph revealed an enlarged cardiac silhouette with increased pulmonary vascularity without pneumonia. Given the presence of hepatomegaly, an abdominal ultrasound (US) (Fig. 1) was performed, which revealed cavernous transformation in the liver with the intralesional massive turbulent vascular flow. Surprisingly, the portal vein was slightly thickened, and the left, middle, and right hepatic veins were significantly dilated.
Abdominal CT scan(Fig. 2) revealed an enlarged liver and multiple patchy hypodense foci in the liver. After contrast medium injection, the lesions showed an enhanced signal intensity. There are multiple arteries in the lesion that were connected to the portal and hepatic veins respectively. The thickened right internal thoracic artery was connected to the left branch of the portal vein.
The patient was diagnosed with PH secondary to CHH due to the enhanced pulmonary blood flow from the congenital hepatic vascular shunt. The newborn was treated with diuretics and fluid restriction. Serial echocardiograms were performed to follow the improvement in PH. However, on serial echocardiograms, pulmonary arterial pressure showed an increasing trend (88 mmHg-110 mmHg-117 mmHg) (Fig. 3). At this crucial juncture, the benefits and risks of arterial angiography and embolization were considered, and a decision was made to embolize of the major vascular feeders. Informed consent was acquired from the parents with the understanding that the major indications for this procedure were PH and CHH at 20 days after birth. Preoperative preparation included an electrocardiogram, an abrosia diet, and total parenteral nutrition.
Under general anesthesia, the innominate artery was accessed under ultrasonic guidance, and a 4F elbowed catheter was advanced into the right internal thoracic artery to the anterior bifurcation of the liver tumor vessels. Gelatin sponge particles were slowly from the right internal thoracic artery to the blind end(Figs. 4). A 4F straight flush catheter was advanced into the proper hepatic artery via femoral arterial-celiac trunk access. After angiography, digital subtraction angiography (DSA) was used to guide selective catheterization and gelatin sponge embolization of the right and left hepatic arteries. Branches at all levels were embolized (Figs. 5). The patient was transferred to the neonatal intensive care unit after surgery.
During the postoperative recovery stage, the newborn gradually stabilized. Abdominal scans revealed a progressively smaller hepatic mass. On serial echocardiograms, pulmonary arterial pressure showed a decreasing trend with normal pulmonary arterial pressure (117 mmHg-59 mmHg-33 mmHg) (Figs. 6) 3 weeks after surgery. Serum α-fetoprotein levels dropped to normal limits during the neonatal period, and BNP levels showed a significant reduction. To enhance the involution of the CHH, the patient was treated with oral propranolol 3 weeks after surgery. The dose of propranolol was increased to a maximum dose of 0.5 mg/kg/day, once every 3 to 4 days to a maximum dose of 2 mg/kg/day. Post-discharge follow-up visits confirmed a continued response to treatment at the time of writing.