A Neonate With Bilateral Pulsating Axillary Arteries and Precordial Pulsations

A 37 weeks’ gestational age male newborn was admitted to the neonatal intensive care unit with respiratory distress soon after birth and developed bilateral axillary artery pulsations and precordial pulsations at 3 days of age.Born to 23-year-old gravida 1 woman with negative prenatal laboratory screens.Fetal ultrasonography at 20 weeks’ gestation showed a solitary intracardiac echogenic focus in the left ventricle. The rest of the fetal survey is normal.Ultrasonography at 31 weeks’ gestation showed polyhydramnios and a well-defined hypoechoic vascular lesion measuring 6.8 cm × 6.7 cm in the lower pole of the placenta in the posterior wall (Figure 1).Delivered at 37 weeks’ gestation by emergency cesarean section because of meconium-stained fluid.Apgar scores are 8 and 10 at 1 and 5 minutes of age, respectively.No maternal fever or prolonged rupture of membranes, unknown Group B Streptococcus.At admission, the infant had minimal subcostal retractions, oxygen saturation of 93% in room air, and visible precordial pulsations. He then developed mild respiratory distress with desaturations and thus was placed on 1 L nasal cannula, and a chest radiograph was obtained (Figure 2). He was started on intravenous antibiotics for suspected sepsis in the setting of respiratory distress. Following, an echocardiography on the same day showed an enlarged right atrium, right ventricle, and left atrium and features suggestive of moderate pulmonary hypertension prompting initiation of oral sildenafil. He developed thrombocytopenia on day 2 of admission.At 3 days of age, he developed bilateral axillary artery pulsations in addition to precordial pulsations (Video 1).Heart rate: 110 beats/minRespiratory rate: 42 breaths/minBlood pressure: 64/38 mm Hg (mean: 42 mm Hg)Oxygen saturation: 98% with nasal cannula at 1 L/minTemperature: 97.8 F (36.6 C)Birth weight: 2.040 kg (less than 3rd percentile); head circumference: 31 cm (less than 3rd percentile); length: 46 cm (less than 10th percentile)General: irritable, but consolableHead: normal shape with wide anterior fontanelleFace: no atypical facial featuresOral cavity: normal, no cleft palateSkin: dry, no lesionsLungs: normal breath sounds equal on both sides with mild subcostal retractionsCardiovascular: normal S1–S2, no gallop, and no murmur; precordial pulsations and bilateral axillary artery pulsations seenAbdomen: soft, no organomegalyNeurologic: irritable, but consolable; jitteriness noticed intermittentlySpine: normalGenitourinary: normalWhite blood cell count (newborn day): 8.7 × 103/μL (8.7 × 109/L)Hemoglobin (newborn day): 16.2 g/dl (160 g/L)Platelet count: 180 × 103/μL (180 × 109/L) on newborn day; decreased to 64 × 103/μL (64 × 109/L) at 6 days of agePeripheral blood smear on day 1 of age: nucleated red blood cells 50/100 white blood cells and moderate thrombocytopenia; repeat smear was done due to decreasing trend in platelet count. It showed thrombocytopenia with eosinophilia and monocytosis with differentials; band cells 2%, neutrophils 60%, lymphocytes 18%, eosinophils 8%, and monocytes 12%C-reactive protein (newborn day): negativeSerum bilirubin (3 days of age): 14.7 mg/dL (251.42 μmol/L)Serum calcium: 9.7 mg/dL (2.42 mmol/L) on newborn day; decreased to 8.1 mg/dL (2.02 mmol/L) at 2 days of age. Vitamin D3: 9.3 ng/ml (23.25 nmol/L). ionized calcium repeated after calcium correction was 5.07 mg/dl (1.27 mmol)Prothrombin time: 17.4 seconds; partial thromboplastin time: 38 seconds; international normalized ratio: 1.5 at admissionElectrolytes (3 days of age): sodium 146 meq/L (146 mmol/L), potassium 5.1 meq/L (5.1 mmol/L), chloride 111 meq/L (111 mmol/L), bicarbonate 19 meq/L (19 mmol/L), serum creatinine 0.88 mg/dL (77.79 μmol/L),Blood culture sterileElectrocardiogram (Figure 3) and repeat echocardiography (Figure 4) were obtained on postnatal day 3Abdominal ultrasonography was also obtained on postnatal day 3 to assess for an arteriovenous malformation, which was normal except for a small paracalyceal cyst (7 × 5 × 4mm) in the lower pole of the right kidneyAortic valve insufficiencyHyperdynamic circulation secondary to arteriovenous malformationPatent ductus arteriosusSubchorionic hematomaTruncus arteriosusThe infant’s chest radiograph on the newborn day (Figure 2) shows cardiomegaly. The infant’s electrocardiogram (Figure 3) reveals a normal rhythm, axis, and ventricular forces without any signs of electrical alternans. The echocardiography shows mild pulmonary hypertension, left ventricular dysfunction, and pulsus alternans (Figures 4 and 5), with the left ventricular outlet showing alternating high- and low-velocity time integrals; there was no pericardial effusion. These findings are consistent with a hyperdynamic circulation associated with high output cardiac failure from the arteriovenous shunting within the placental chorioangioma. The infant was given intravenous calcium at 2 days of age and dobutamine infusion to improve left ventricular function, and the pulsus alternans resolved after 24 hours of treatment. There were absent precordial pulsations at 5 days of age.The infant’s thrombocytopenia improved spontaneously. Brain magnetic resonance imaging was done at 4 days of age because of excess irritability with jitteriness, and results were normal. An electroencephalogram was also obtained on day 3 because of irritability and showed intermittent generalized epileptiform sharp spikes concerning for a possible seizure, and he received a loading dose of intravenous phenobarbital followed by maintenance intravenous phenobarbital. He was discharged on oral phenobarbital, which was tapered and stopped during follow-up. The cause of the subclinical seizure was not identified and thought to be a possible result of hypocalcemia. A small pericalyceal cyst was an incidental finding.Placental chorioangioma is the most common benign placenta tumor, arising from the chorionic mesenchyme.1 It is seen in 0.41% to 1.4% of examined placentas by microscopy.1 A larger tumor (greater than 4 cm) is associated with complications in the pregnant person and fetus, with a prevalence of large tumors ranging from 1 in 9000 to 1 in 50 000 pregnancies.1 The size and vascularity of the tumor determine the shunt volume, correlating with the impact on the fetus and newborn.Complications of placental chorioangiomas in pregnant persons include preeclampsia, antepartum hemorrhage, maternal mirror syndrome (defined as maternal edema associated with fetal hydrops), and preterm labor.2 Polyhydramnios occurs as a result of high urine output secondary to the hyperdynamic circulation in the fetus and transduction of fluid from the tumor surface. A large tumor is characterized by significant arteriovenous shunting that leads to high-output cardiac failure in the fetus. Fetal anemia and thrombocytopenia can occur because of hematologic sequestration within the tumor. The other fetal complications of a large placental chorioangioma include fetal growth restriction, fetal cardiomegaly, and fetal hydrops.2 In severe cases, intrauterine fetal demise can occur.Placental chorioangiomas are frequently located near the cord insertion site, bulging into the amniotic cavity with the umbilical artery serving as the feeding vessel. They are diagnosed by ultrasonography and color Doppler by identifying the same pulsatile flow in the feeding vessel as that of the umbilical artery; low resistance flow may also be present within the mass as a result of the arteriovenous shunt.Fetal intervention of placental chorioangiomas is based on the presence of complications in the pregnant person and fetus. Supportive intrauterine treatment includes transfusions for fetal anemia, oral indomethacin for polyhydramnios, and digoxin administration to the pregnant person if there is fetal cardiomegaly. Definitive intrauterine treatment aims to block the arteriovenous shunt, which includes alcohol injection,3 endoscopic laser coagulation, and interstitial laser therapy.4 Laser photocoagulation can be performed either fetoscopically or by ultrasonography-guided interstitial laser therapy.5 In one study, the fetoscopic approach was used for a posterior tumor and interstitial laser therapy was used for an anterior tumor.6Several case reports of the effects of placental chorioangioma on the neonate have been published. Panat et al described a neonate with a prenatal finding of a large placental chorioangioma, who presented with cardiac failure leading to renal failure, disseminated intravascular coagulopathy, and severe pulmonary hypertension.5 Tom et al found an association of neonatal cardiomyopathy with multiple small placental chorioangiomas, which produced an effect similar to giant placental chorioangioma; the neonate improved with supportive management.7 Ma et al found that one-third of large placental chorioangiomas developed severe complications of the pregnant person or fetus and that one-fifth of fetuses had intrauterine demise; the authors also noted that the long-term prognosis for surviving fetuses is good.1Postnatally, infants with intrauterine placental chorioangioma can present with high-output cardiac failure. Typical clinical findings are tachypnea, tachycardia, and precordial pulsations. Axillary artery pulsations can be found in some patients with this hyperdynamic state. Pulsus alternans can also be found in infants with severe left ventricular dysfunction and can be diagnosed by echocardiography showing alternating high- and low-velocity time integrals of left ventricular output in spectral Doppler mode. The 2 mechanisms implicating in the development of pulsus alternans are Alternating force of left ventricular contraction due to variation in the end-diastolic volume, andAbnormal calcium cycling resulting from overexpression of calsequesterin.8The high-output cardiac failure that occurs with placental chorioangioma results from low peripheral vascular resistance from arteriovenous shunting. The treatment for high-output cardiac failure includes restriction of fluids, diuretics, and inotropic support; in patients with associated pulsus alternans, improvement occurs as left ventricular function improves.Aortic valve regurgitation in adults can have bounding pulses and visible peripheral artery pulsations. Congenital bicuspid aortic valve is usually asymptomatic, and symptoms are related to development of aortic stenosis, aortic insufficiency, or both. Patients with a patent ductus arteriosus can present with hyperdynamic circulation such as bounding pulses, wide pulse pressures, and precordial pulsations, but the symptoms do not typically present immediately after birth. Patients with truncus arteriosus may present with bounding peripheral pulses due to excessive runoff into the pulmonary arteries. However, these infants exhibit respiratory distress and persistent cyanosis despite oxygen therapy. A subchorionic hematoma lacks internal vascularity and thus is not consistent with the placental findings in this case.SummaryA large placental chorioangioma can be associated with complications in the pregnant person and fetus.The complications seen in neonates due to large placental chorioangioma are high-output cardiac failure and pulmonary hypertension.The long-term prognosis of surviving neonates with placental chorioangioma is good.Pulsus alternans is a feature of severe left ventricular dysfunction; the prognosis depends on the etiology.American Board of Pediatrics Neonatal-Perinatal Content SpecificationUnderstand potential outcomes of precordial pulsation in obstetrics and maternal-fetal medicineI thank Sharmila Senthil Kumar, MD(OG), DRM, Starmed Hospital, Salem; Sasi Anand, DCH, DNB, Ramalingam Hospital, Salem; Raeshmi Ramalingam, MD(paediatrics), Ramalingam Hospital, Salem; and Shatish Kumar, MD, DNB, consultant cardiologist, SKS Hospital, Salem for assistance with this patient’s diagnosis and management.