Infantile Hepatic Hemangiomas and Hemoglobin SC Disease in a Very Low Birth Weight Premature Infant with Pulmonary Hypertension and Congestive Heart Failure: A Case of Multifactorial Etiology

To assess the efficacy of transcatheter arterial embolization (TAE) plus propranolol treatment for infantile hepatic hemangioma (IHH). A retrospective study of symptomatic IHH and hemodynamic changes in IHH was conducted between 2016 and 2019. There were five boys and seven girls with diffuse lesions (n = 7) and multifocal lesions (n = 5). Hepatomegaly and abdominal distension (n = 6) were the predominant clinical presentations. Seven patients (58.3%) had multiple cutaneous hemangiomas. Pulmonary arterial hypertension, heart failure (n = 4), and hypothyroidism (n = 4) were observed. A total of 17 TAE procedures were performed in 12 IHH cases, with a technical success rate of 100%. All patients received standard propranolol orally, and one patient was orally administered metacortandracin. Two patients died of heart failure and multiple organ dysfunction caused by an enlarged liver. In addition, one patient was not reexamined after discharge. Of the remaining nine children, the average follow-up time was 10.78months (range 2-28months), and they all responded well to TAE combined with oral propranolol. TAE combined with propranolol is safe and effective for the treatment of IHH, demonstrating low complication rates.

A New Classification of Pulmonary Hypertension

Unusual Case of Hypothyroidism in an Infant With Hepatic Hemangioma

Objective To discuss the effect of the occurrence of congestive heart failure on the outcome of pregnant women with pulmonary hypertension. Methods Fifty-four pregnant patients complicated with pulmonary hypertension were admitted from January 2000 through December 2010. Among them, 34 had comorbidity of congestive heart failure. The timing and mode of pregnancy termination, and perinatal outcomes were studied, and comparison was made between those with and without heart failure. Results ① Of all 54 pregnant women with pulmonary hypertension, 34 had congestive heart failure. The incidences of congestive heart failure in patients with mild, moderate and severe degree of pulmonary hypertension were 27.78% (5/18), 73.33% (11/15) and 85.71% (18/21), respectively (P<0.05).②The rate of maternal complications was 47.06% (16/34) and maternal mortality was 17.65% (6/34) in the patients with combined pulmonary hypertension and heart failure. The rate of iatrogenic fetal loss was 29.41% ( 10/34) , preterm labor 52.94% (18/34), neonatal asphyxia 35.29% (12/34) and neonatal mortality 23.53% (8/34) in case of patients with pulmonary hypertension complicated with congestive heart failure. ③The rate of Cesarean section was 91. 18% (31/34) in the patients with combined pulmonary hypertension and heart failure. ④ The rates of iatrogenic induction, premature delivery, maternal complications and mortality, neonatal asphyxia and fetal or neonatal fatality were significantly higher in women with combined pulmonary hypertension and heart failure than those with simple pulmonary hypertension ( P < 0. 05). Conclusions The risk of heart failure increases with the severity of pulmonary hypertension. The occurrence of heart failure is the most important factor affecting the outcome of patients in pregnancy already complicated with pulmonary hypertension , and Cesarean section is the safer mode of termination of pregnancy in this cohort of women. Key words: Pregnancy complications; Pulmonary arterial hypertension; Heart failure; Perinatal outcomes

Atrial septal defect (ASD) occlusion in adult patients with advanced age and left or right heart diastolic or systolic heart failure and in patients with pulmonary arterial hypertension bears a considerable risk of deterioration of heart failure. Therefore, we conducted this feasibility trial in 15 ASD patients with pulmonary hypertension and/or right heart failure using a fenestrated Amplatzer septal occluder (AGA Medical Corporation, Golden Valley, MN), allowing an overflow of blood in both directions in case of univentricular diastolic or systolic heart failure. In all patients, the device could be implanted without complications. All symptomatic patients showed an improvement in the New York Heart Association (NYHA) class, and no right or left heart decompensation occurred. On echocardiography, right ventricular end diastolic dimension (RVEDD) and pulmonary artery pressure (PAP) decreased significantly, whereas left ventricular end diastolic dimension (LVEDD) increased. Our series of 15 patients with fenestrated ASD occlusion shows that high-risk ASD occlusion can safely be accomplished with excellent clinical results and without complications by a fenestrated occluder.

Task Force 7: Pediatric Cardiology Fellowship Training in Pulmonary Hypertension, Advanced Heart Failure, and Transplantation. SPCTPD/ACC/AAP/AHA.

Improved survival of very low birth weight (VLBW) premature infants requires urgent intensive care, professional nursing and medical care. On the other hand, long hospital stay period imposes emotional and economic burdens on the family and society. Therefore, it is necessary to clarify the most important factors affecting their hospitalization duration to lessen unwanted outcomes of premature birth and to eliminate or relieve the problems. In a descriptive-analytical study, 170 low birth weight premature infants were assessed in a regular daily follow-up in Alzahra Hospital, Tabriz, Iran. Probable factors affecting NICU (neonatal intensive care unit) stay, such as birth age and weight, time of trophic feeding initiation, time of regain birth weight, and duration of parenteral nutrition, were evaluated. Data was analyzed using descriptive statistics and linier regression model in SPSS14. Statistical significance was considered at 0.05. Mean birth weight and age of infants were 1310.26 ± 804.26 g and 30.51 ± 0.34 weeks, respectively. Duration of NICU stay was 14.51 ± 10.12 days. Intrauterine growth retardation, positive C-reactive protein (CRP) and blood culture during hospitalization stay, bloody or bile stained gastric remnants, days to regain birth weight, and total enteral nutrition intolerance were the most important factors affecting NICU stay among the studied infants. It seems that eliminating risk factors of intrauterine growth retardation during pregnancy, preventing perinatal and nosocomial infections and taking suitable and tolerable feeding strategies will be effective on NICU stay duration in low birth weight premature infants. Therefore, required policies, especially early trophic feeding, are suggested to eliminate the existing difficulties.

A successful first-line treatment with propranolol of multifocal infantile hepatic hemangioma with high-flow cardiac overload is described

Commentary: Truncus among us

Infantile hepatic hemangioma (IHH) is a common liver tumor in infants and shares the same characteristics as cutaneous infantile hemangioma (IH). Propranolol is effective for symptomatic IHH. The clinical features between cutaneous IH and IHH, and treatment efficacy of IHH (smaller than 4 cm) is unclear. To evaluate the correlation of clinical features between cutaneous IH and IHH, as well as efficacy of systemic propranolol in the treatment of cutaneous IH combined with IHH. The clinical data of infants with complicated cutaneous IH combined with IHH treated with systemic propranolol (1.5 ~ 2 mg/(kg d)) from January 2011 to October 2020 were retrospectively analyzed. Forty-five cases with IHH combined with complicated cutaneous IH were reviewed. Single cutaneous IH is more likely to be combined with focal IHH, cutaneous IH greater than 5, more likely to be combined with multiple IHH (Pearson = 0.546, p < 0.01). The mean age of focal and multiple IHH regression was 11.93 ± 14.42 months and 10.20 ± 9.15 months, respectively. The number of cutaneous IH were correlated with the number of IHH. There was no difference in the age of complete remission for focal and multiple IHH.

Objective To investigate the treatment of symptomatic patent ductus arteriosus (PDA) in very low birth weight preterm infants. Methods From January 1, 2008 to December 31, 2010, 78 very low birth weight preterm infants (birth weight＜1500 g) were diagnosed as symptomatic PDA. Among which, 42 cases administered orally with indomethacin (0.2 mg/kg, every 12 hrs for three times) were taken as treatment group, while five cases in this group who failed to indomethacin treatment were interrupted with video-assisted thoracoscopic surgery. And 36 cases who did not receive treatment for ductus arteriosus were taken as control group. The clinical outcomes, complications and prognosis of these patients were observed. Results There were no significant differences between the gentle percentage, gestational age, diameter of ductus arteriosus, rate of complicated with heart failure, sepsis, neonatal respiratory distress syndrome and intraventricular hemorrhage of two groups (P>0.05, respectively). The ductus arteriosus closed in 33 patients of treatment group (78.6%) and in nine patients of control group (25.0%)(χ2=22.39,P=0.000). There were no significant differences in serum creatinine level and platelet count between before and after the treatment in treatment group(P>0.05). Compared with control group, the treatment group had lower incidence of intraventricular hemorrhage (z=1.167, P=0.030), shorter duration of oxygen therapy [(8.0±5.5) d vs (13.3±9.3) d, t=2.225, P=0.032] and shorter hospital stay [(39.0±7.7) d vs (43.6±10.6) d, t=2.229, P=0.029]; while the incidence of bronchopulmonary dysplasia and necrotizing enterocolitis were similar (P>0.05). The five cases of PDA who received video-assisted thoracoscopic surgery were successfully interrupted with no residual shunt left, while three of them had lung infections and one had pleural effusion, but no pneumothorax and infant death associated with surgery occurred. Conclusions Symptomatic PDA of very low birth weight preterm infants should be treated actively. Oral indomethacin was an effective and safe method to cure the PDA in these infants. Surgical ligation under video-assisted thoracoscopic surgery after failure of indomethacin treatment might be a good option. Key words: Infant, premature; Infant, very low birth weight; Ductus arteriosus, patent; Indomethacin; Thoracoscopy

<i>Circulation</i> Editors’ Picks

Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France.

Neonates are at risk of developing hypernatremic dehydration and its associated complications, such as stroke, dural sinus thrombosis and renal vein thrombosis. Pulmonary hypertension has not been described as a complication of hypernatremia. We report a case of a seven-day-old neonate with severe hypernatremic dehydration who went on to develop pulmonary hypertension and right heart failure needing extracorporeal membrane oxygenation (ECMO). Normal or high anion gap metabolic acidosis commonly accompanies hypernatremic dehydration. The presence of acidosis and/or hypoxia can delay the normal drop in pulmonary vascular resistance (PVR) after birth, causing pulmonary hypertension and right ventricular failure. A high index of suspicion is paramount to diagnose pulmonary hypertension and aggressive correction of the acidosis and hypoxia is needed. In the presence of severe right ventricular failure, ECMO can be used as a bridge to recovery while underlying metabolic derangements are being corrected.

Pulmonary Hypertension in Heart Failure With Preserved Ejection Fraction: A Community-Based Study