Role of functional echocardiographic parameters in the diagnosis of bronchopulmonary dysplasia-associated pulmonary hypertension.

Echocardiogram (echo) is a commonly used noninvasive modality for the diagnosis of bronchopulmonary dysplasia associated pulmonary hypertension (BPD-PH). Though not considered the gold standard for the diagnosis of BPD-PH, it is an extremely valuable tool in the neonatal and pediatric population, especially when cardiac catheterization is not feasible. In addition to the traditional echo parameters that are used to assess the presence of BPD-PH, much attention has been recently placed on newer bedside echo measures, the so-called functional echo parameters, to aid and assist in the diagnosis. This review article provides a brief introduction to BPD-PH, describes the pitfalls of traditional echo parameters and details the newer echo modalities currently available for the diagnosis of neonatal PH.

Benign Structures Mimicking Right Atrial Masses on Prenatal Ultrasound.

Advances in imaging have resulted in more frequent reporting of primitive right atrial structures which can sometimes mimic cardiac tumors in prenatal ultrasound. Prominent crista terminalis and Chiari network are examples of these structures. We describe two cases of pregnant women referred to the fetal cardiology clinic for fetal echocardiography for right atrial masses seen on prenatal ultrasound suspicious of tuberous sclerosis. The first case subsequently diagnosed as crista terminalis and the second case as a prominent Chiari network. Postnatal ECHO confirmed the benign nature of these structures. It is important to differentiate tumors from prominent benign structures in the right atrium in fetal ECHO. The location and the similar echogenicity to the adjacent atrial tissue are clues for differentiation of these structures from atrial tumors.

Urinary NT-proBNP as a potential noninvasive biomarker for screening of pulmonary hypertension in preterm infants: a pilot study.

OBJECTIVE: This pilot study aimed to determine the feasibility of urinary NT-proBNP (NT-proBNP) as a potential noninvasive screening marker for pulmonary hypertension (PH). STUDY DESIGN: A prospective cross-sectional study was conducted. Preterm infants (PI) (birthweight <1500 gm and <30 weeks gestational age (GA)) were enrolled. Serial urinary NT-proBNP measurements and echocardiograms (ECHO) were performed at 28, 32, and 36 weeks. RESULTS: Thirty-six patients were included in the final analysis (BPD-PH group = 6, BPD group = 20, control = 10). Urinary NT-proBNP levels were higher in the BPD-PH group compared with BPD and control groups at all study intervals. A urine NT-proBNP cutoff level of 2345 pg/ml at 28 weeks of GA had a sensitivity and specificity of 83.3% and 84.2%, respectively, for detection of BPD-PH (AUC 0.816, p = 0.022). CONCLUSION: Urinary NT-proBNP measurement is feasible in preterm infants and appears to be a good noninvasive screening tool for PH.

Asymptomatic Right Ventricular Hypoplasia in Twin Siblings: A Normal Variant or Cause of Early Mortality?

Right ventricular (RV) hypoplasia may develop secondary to pulmonary or tricuspid valve atresia. These patients are usually symptomatic early in life and need prompt intervention. Isolated RV hypoplasia is a rare congenital heart disease. We report a case of 23-year-old twins who have been monitored for the last 14 years for isolated right ventricular hypoplasia. ECHO and MRI studies showed a small, heavily trabeculated, nonapex-forming RV and mild tricuspid valve insufficiency. The girl has a patent foramen ovale (PFO). Otherwise, the cardiac anatomy and function was normal. They have both been completely asymptomatic from the cardiac standpoint. The family history is remarkable for death of father at the age of 30 years with autopsy suggestive of a hypoplastic RV. The paternal uncle also died at the age of 46 years, and his son has an unidentified congenital heart disease. The family history appears to suggest an autosomal dominant pattern of inheritance with variable expressivity. However, the chromosome microarray analysis of the twins did not identify any variations of clinical significance.

Antenatal Magnesium Sulfate Exposure and Hemodynamically Significant Patent Ductus Arteriosus in Premature Infants.

Objective The use of antenatal magnesium sulfate (MgSO 4 ) has been associated with neuroprotective effects. One of its' proposed mechanisms of action includes antagonism of calcium channels. Calcium influx is important for closure of ductus arteriosus. We hypothesized that antenatal MgSO 4 exposure may be associated with an increased risk of hemodynamically significant patent ductus arteriosus (HsPDA) in premature infants (PI). Study Design A prospective cohort study conducted in two parts. PI (< 32 weeks and < 1,500 g) were recruited ( n = 105). All infants had Echocardiograph (ECHO; within 3 days) and blood samples drawn at the same time for B-type natriuretic peptide (BNP; part 1) and NTproBNP (N-terminal pro BNP; part 2) measurements. HsPDA was defined as a PDA diameter > 1.5 mm and BNP levels > 40 pg/mL or NTproBNP > 10,200 pg/mL. Infants were divided into two groups based on antenatal MgSO 4 exposure. Data were analyzed using SPSS 23. Difference in baseline characteristics and antenatal steroid use in the two groups was analyzed. A matched group analysis was performed to adjust for the difference in the numbers between the two groups. A p -value < 0.05 was considered significant. Results There was no significant difference seen in baseline characteristics or use of antenatal steroids in exposed versus unexposed ( n = 95 vs. n = 10). There was a significant negative correlation between antenatal MgSO 4 exposure and HsPDA in PI ( p ≤ 0.05). However, this association was not significant after matched group analysis. Conclusion Antenatal MgSO 4 exposure is not associated with an increased risk of HsPDA. It may be associated with a decreased likelihood of HsPDA.

Prenatal Diagnosis and Successful Palliation of Absent Aortic Valve with Hypoplastic Left Heart Syndrome: A Case Report and Review of Literature.

Introduction Congenital absence of the aortic valve leaflets is a rare association with hypoplastic left heart syndrome (HLHS). Case A 37-year-old pregnant woman was referred for fetal evaluation of possible HLHS at 22 weeks of gestation. The fetal echocardiogram (ECHO) was remarkable for a hypoplastic left atrium, nearly atretic mitral valve, small left ventricle, and a hypoplastic aortic valve with severe aortic insufficiency. A female infant was born at term and postnatal ECHO confirmed the above findings. In addition, there was complete absence of the aortic valve leaflets. The patient underwent Norwood's procedure at day 5 of life with atrial septectomy, over-sewing of the aortic valve annulus, and a 4 mm Sano's shunt between the right ventricle and the main pulmonary artery. She tolerated this surgery well and subsequently underwent a bidirectional Glenn's procedure at 8 months of life. Conclusion Prenatal diagnosis of absent aortic valve should be suspected in the presence of severe aortic insufficiency in the fetal ECHO. Early postnatal intervention is critical as those patients are likely to deteriorate quickly. The over-sewing of the aortic valve may be important to prevent coronary steal and myocardial hypoperfusion which could potentially be detrimental.

Left Ventricular Noncompaction in a Child with Turner Syndrome.

Congenital heart disease (CHD) may cause a significant comorbidity in patients with Turner syndrome. The commonly reported CHD in these patients includes bicuspid aortic valve and coarctation of the aorta. Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy that has been reported in literature only three times in adult patients with Turner syndrome. We report the first case of a 6-year-old asymptomatic female with Turner syndrome who was referred for cardiac evaluation after her Turner syndrome diagnosis. Echocardiogram was suspicious for LVNC, which was confirmed on cardiac magnetic resonance imaging.

Sildenafil Use in the Treatment of Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension: A Case Series.

Objective This article studies the role of sildenafil in reducing myocardial stress (measured by serial N-terminal pro b-type natriuretic peptide [NTproBNP] levels) secondary to bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH). Study Design This is a case series of three extremely low birth weight infants with severe BPD at 36 weeks' postmenstrual age. All infants had very elevated NTproBNP (> 2,000 ng/dL) levels and echocardiographic evidence of BPD-PH. Sildenafil was started and infants were followed up every 2 weeks clinically along with NTproBNP levels and echocardiograms. Results After 4 weeks of sildenafil treatment, NTproBNP levels decreased significantly in all infants, echocardiograms showed significant improvement in one infant, and respiratory severity score improved significantly in one infant. All infants tolerated sildenafil. Conclusion Sildenafil reduced NTproBNP levels in all infants with BPD-PH but the echocardiographic findings and respiratory scores did not improve consistently. We speculate that this may be due to a delay in diagnosis and initiation of therapy after irreversible pulmonary changes have set in.

NTproBNP as a surrogate biomarker for early screening of pulmonary hypertension in preterm infants with bronchopulmonary dysplasia.

OBJECTIVE: Pulmonary hypertension (PH) is a known complication of bronchopulmonary dysplasia (BPD). This study aimed to determine the utility of serial N-Terminal pro-Brain Natriuretic Peptide (NTproBNP) levels in the screening of BPD associated PH (BPD-PH) in preterm infants. STUDY DESIGN: Infants with birth weight <1500 g and <30 week corrected gestational age (CGA) were followed with serial NTproBNP levels and echocardiograms (ECHO). They were divided into control, BPD and BPD-PH groups. Statistical analyses included repeated measures analysis of variance and receiver operator curve (ROC) generation. RESULTS: Infants in the BPD-PH and BPD group had significantly elevated NTproBNP levels as compared to the control group. ROC curves for NTproBNP at 28 weeks CGA provided a cut-point of 2329 pg/ml and 578.1 pg/ml for detection of BPD-PH and BPD, respectively. CONCLUSIONS: NTproBNP appears to be a good screening tool to determine the onset of BPD-PH as early as 28 weeks CGA.

High-sensitivity troponin T in preterm infants with a hemodynamically significant patent ductus arteriosus.

OBJECTIVE: To investigate if a hemodynamically significant patent ductus arteriosus (hsPDA) leads to elevated high-sensitivity troponin T (hsTnT) and NTproBNP levels in serum. STUDY DESIGN: Infants <34 weeks and <1500 g were prospectively enrolled, except those with major congenital or chromosomal anomalies. An echocardiogram (ECHO) was performed and hsTnT and NTproBNP were measured within 5 days of life and repeated after treatment of hsPDA. Clinical, ECHO, and hsTnT data were analyzed using Student t-test, two proportion z-test, and regression analysis. RESULTS: Seventy infants were enrolled. Infants in the hsPDA group had lower gestation and birth weight. Mean hsTnT and NTproBNP levels in the hsPDA group were higher compared to the group without an hsPDA, with levels being 251.54 vs 161.6 pg/ml, p < 0.01 for hsTnT and 18181.02 vs 3149.23 pg/ml, p < 0.001 for NTproBNP. CONCLUSION: HsPDA leads to increased hsTnT and NTproBNP levels in preterm infants without affecting cardiac function.

An Unusual Mechanism of Closure of Muscular Ventricular Septal Defects.

Ventricular septal defects (VSDs) are the most common congenital heart defects. Most of the small or moderate size (<6 mm) muscular VSDs close spontaneously within the first two years of life. The usual mechanism of spontaneous closure involves muscular tissue encroachment with superimposed fibrosis or primary fibrous tissue formation around the margins of the defect. We describe an unusual mechanism of spontaneous closure of a muscular VSD.

Coarctation of the Aorta as a Complication of Surgical Ligation of Patent Ductus Arteriosus in a Premature Infant.

Surgical ligation of a patent ductus arteriosus (PDA) is a commonly performed procedure. Complications are infrequent and most commonly include recurrent laryngeal nerve injury and rarely ligation of left pulmonary artery. We report a case of accidental ligation of the descending thoracic aorta leading to a clinically significant coarctation.

Dilated Cardiomyopathy Induced by Chronic Starvation and Selenium Deficiency.

Protein energy malnutrition (PEM) has been rarely documented as a cause of cardiovascular abnormalities, including dilated cardiomyopathy. Selenium is responsible for antioxidant defense mechanisms in cardiomyocytes, and its deficiency in the setting of PEM and disease related malnutrition (DRM) may lead to exacerbation of the dilated cardiomyopathy. We report a rare case of a fourteen-year-old boy who presented with symptoms of congestive heart failure due to DRM and PEM (secondary to chronic starvation) along with severe selenium deficiency. An initial echocardiogram showed severely depressed systolic function consistent with dilated cardiomyopathy. Aggressive nutritional support and replacement of selenium and congestive heart failure medications that included diuretics and ACE inhibitors with the addition of carvedilol led to normalization of the cardiac function within four weeks. He continues to have significant weight gain and is currently completely asymptomatic from a cardiovascular standpoint.

Junctional Bradycardia as Early Sign of Digoxin Toxicity in a Premature Infant with Congestive Heart Failure due to a Left to Right Shunt.

Introduction Congestive heart failure due to left to right cardiac shunt is usually managed medically with diuretics, angiotensin converting enzyme inhibitors, and, in some cases, with the addition of digoxin. Case We report a 31-week gestation premature male infant who did not respond to such treatment and developed hyperaldosteronism and severe hypokalemia secondary to activation of the renin angiotensin aldosterone system. The hypokalemia was not responsive to intravenous KCL supplementation and induced digoxin toxicity despite a relatively normal digoxin level. The earliest signs of digoxin toxicity in the patient were junctional rhythm and bradycardia. The discontinuation of digoxin and the administration of digoxin specific immunoglobulin fragments (Fab) reversed those changes. The addition of spironolactone (an aldosterone antagonist) had a dramatic effect, resulting in clinical improvement of the patient coupled with normalization of Q4 serum and urine electrolytes. Conclusion Serum Digoxin level alone may fail as an independent guide in the diagnosis of digoxin toxicity when hypokalemia is present. In premature infants with congestive heart failure and hypokalemia, addition of an aldosterone antagonist should be considered.

Unusual presentation of unroofed coronary sinus with cyanosis after ventricular septal defect closure.

Cyanosis after surgical closure of ventricular and/or atrial septal defect is very unusual. We report a case of unroofed coronary sinus in a six-month-old boy who developed cyanosis after surgical closure of ventricular and atrial septal defects. Clinical presentation, diagnosis, and management are discussed.

Successful embolization of a large vein of galen malformation in a premature infant presenting with congestive heart failure and persistent pulmonary hypertension.

We report a case of a premature infant with a vein of Galen malformation (VGM) who presented with high-output heart failure, pulmonary hypertension, and respiratory distress. An echocardiogram showed normal cardiac anatomy, patent ductus arteriosus, and retrograde flow in the descending aorta. Ultrasonography and computed tomography of head confirmed the diagnosis of a large VGM. The patient developed multiorgan failure. Endovascular embolization of the VGM was successfully performed with an excellent outcome.

Absent pulmonary valve syndrome with left hemitruncus.

Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect, usually associated with tetralogy of Fallot (TOF), although other associations have been described. A pregnant woman was referred to fetal echocardiography clinic from the Maternal Fetal Medicine department due to abnormal findings on routine antenatal ultrasound, showing a pulsatile cystic mass above the left atrium and a suspected TOF. A fetal echocardiogram confirmed the presence of TOF/APVS. The pulsatile cystic mass was the aneurysmally dilated main pulmonary artery. The exact origin of the left pulmonary artery (LPA) was not clearly established prenatally. A postnatal echocardiogram of the neonate showed an abnormal origin of the LPA from the ascending aorta (hemitruncus). The neonate subsequently underwent surgical repair with a good outcome. We present a novel case of a TOF/APVS associated with an abnormal origin of the LPA from the ascending aorta.

Twin preterm neonates with cardiac toxicity related to lopinavir/ritonavir therapy.

We report twin neonates who were born prematurely at 32 weeks of gestation to a mother with human immunodeficiency virus infection. One of the twins developed complete heart block and dilated cardiomyopathy related to lopinavir/ritonavir therapy, a boosted protease-inhibitor agent, while the other twin developed mild bradycardia. We recommend caution in the use of lopinavir/ritonavir in the immediate neonatal period.