Cardiogenic shock and neonate

Cardiogenic shock (CS) occurs infrequently in pregnancy and has a high mortality rate. Medical treatment options are few, with limited evidence of efficacy. Temporary mechanical circulatory supports (tMCS) may play a key role in addressing this therapeutic lacuna. We report successfully managing second-trimester CS using an Impella 5.5 micro-axial pump. Our patient presented in the second-trimester with CS. Hemodynamic parameters indicated biventricular dysfunction (low cardiac index, low pulmonary artery pulsatility index)...

OBJECTIVES: This study aims to describe the effectiveness of low initial alprostadil dosages to maintain a patent ductus arteriosus (PDA) in infants with ductal-dependent congenital heart disease (DDCHD). Secondary objectives were to describe any adverse drug events, describe prescribing trends, describe ductus arteriosus diameter changes, and compare the safety and efficacy of very low and low initial alprostadil dosage regimens. METHODS: This retrospective observational cohort study at the British Columbia's Women's and Children's Hospital neonatal intensive care unit and pediatric intensive care unit examined neonates admitted with DDCHD who received alprostadil to maintain ductal patency...

Ebstein anomaly (EA) is a congenital dysplasia of the tricuspid valve resulting in reduced right ventricular (RV) volume and tricuspid regurgitation. Severe EA in the neonatal period is associated with high mortality. The Starnes procedure (fenestrated RV exclusion) is reserved for EA patients with cardiogenic shock and has previously committed patients to single ventricle (SV) palliation. In this report, we present the results of a strategy to redirect patients utilizing the Da Silva Cone operation to achieve a 2 or 1...

BACKGROUND: The first clinical manifestations of inherited metabolic diseases occur in the neonatal period in up to half of cases, often with nonspecific symptoms, making their recognition challenging. This study aimed to characterise inherited metabolic disease cases with neonatal presentation requiring admission to the paediatric intensive care unit in a Portuguese reference centre for inherited metabolic diseases. MATERIAL AND METHODS: An observational study with retrospective data collection was performed, including all newborns with an inherited metabolic disease admitted to the pediatric intensive care unit between June 2011 and June 2022...

INTRODUCTION: Undiagnosed congenital heart disease and management of pediatric cardiogenic shock presents a diagnostic challenge for the emergency clinician. These diagnoses are rare and require a high index of suspicion given the overlap with more common pediatric pathology. Point-of-care ultrasound can assist in differentiating these presentations. We present a case of neonatal cardiogenic shock secondary to a previously undiagnosed congenital heart disease, specifically Shone complex, detected using point-of-care ultrasound...

Generalized Arterial Calcifications of Infancy (GACI) is an extremely rare autosomal recessive genetic condition, mostly due to pathogenic variations in the ENPP1 gene (GACI1, MIM # 208000, ENPP1, MIM *173335). To date 46 likely pathogenic or pathogenic distinct variations in ENPP1 have been described, including nonsense, frameshift, missense, splicing variations, and large deletions. Here we report a case of GACI in a male newborn with a homozygous stop-loss variant in ENPP1 treated in Nancy Regional University Maternity Hospital...

BACKGROUND: Early-onset isolated systemic hypertension is a rare condition of unknown genetic origin. Renovascular, renal parenchymal diseases or aortic coarctation are the most common causes of secondary systemic hypertension in younger children and neonates. We investigated the genetic bases of early-onset isolated systemic hypertension. METHODS: Whole-exome sequencing (WES) was followed by variant filtering and Sanger sequencing for validation and familial segregation of selected variants in a large consanguineous family...

BACKGROUND: Using bibliometric method to analyze the research status and development trend of extracorporeal membrane oxygenation (ECMO), we aim to provide clinicians, scientists, and stakeholders with the most up-to-date and comprehensive overview of ECMO research. MATERIALS AND METHODS: Using Excel and VOSviewer, the literature on ECMO was systematically analyzed regarding publication trends, journal source, foundation, countries, institutions, core authors, research hotspots, and market distribution...

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BACKGROUND: Hypoplastic left heart syndrome (HLHS) is a congenital heart disease that is associated with high mortality rates in the early neonatal period and during surgical treatments. This is mainly due to missed prenatal diagnosis, delayed diagnostic suspicion, and consequent unsuccessful therapeutic intervention. CASE REPORT: twenty-six hours after birth, a female newborn died of severe respiratory failure. No cardiac abnormalities and no genetic diseases had been evidenced or documented during intrauterine life...

Supraventricular tachycardia (SVT) is one of the most common conditions in neonates that require emergency cardiac care. Its incidence in infancy is 0.06 and 0.25 per 1000 patients per year by the age of 1 month and one year respectively. The symptoms are usually nonspecific and include poor feeding, irritability, vomiting, cyanosis, and pallid spells. If the symptoms are unrecognized for hours to days, the infant can present with significant hemodynamic compromise or heart failure. Despite the success of conservative management in most cases, catheter ablation is required in cases of failure of medical treatment...

Obstructed total anomalous pulmonary venous connection (TAPVC) represents a true pediatric cardiac emergency. The patient may present in extremis secondary to severe pulmonary hypertension and cardiogenic shock which increases perioperative mortality. We present a neonate who underwent a successful staged hybrid approach for an Infradiaphragmatic obstructed TAPVC.

BACKGROUND: Neonatal Marfan syndrome (nMFS), the most severe form of Marfan syndrome, is a rare condition that presents a clinical and treatment challenge. nMFS has high infant mortality related to progressive valvular dysfunction. Valve replacement in this setting improves long-term prognosis but carries high morbidity and mortality. Thus, sharing clinical experience in treating such patients is valuable. CASE SUMMARY: A 2 year old with nMFS underwent tricuspid valve annuloplasty and prosthetic mitral valve replacement...

Background: The effectiveness of veno-arterial extracorporeal life support (V-A ECLS) in treating neonatal and pediatric patients with complex congenital heart disease (CHD) and requiring cardio-circulatory assistance is well-known. Nevertheless, the influence of left ventricle (LV) distension and its countermeasure, namely LV unloading, on survival and clinical outcomes in neonates and children treated with V-A ECLS needs still to be addressed. Therefore, the aim of this study was to determine the effects of LV unloading on in-hospital survival and complications in neonates and children treated with V-A ECLS...

Bordetella pertussis (B. pertussis)  commonly infects individuals of all ages. However, pertussis, the disease caused by B. pertussis infection, is most severe in young infants. Severe pertussis, defined by the presence of refractory hypoxemia, pneumonia, cardiogenic shock, and hyperleukocytosis, is associated with significant morbidity and mortality. Both hyperleukocytosis and pulmonary hypertension have been found to be predictive of mortality in young infants. Leukoreductive strategies such as leukapheresis and exchange transfusion have been employed to treat these complications...

A term infant girl with uneventful antenatal history had an erythematous rash followed by fever from day 8. She was diagnosed with late-onset sepsis and was treated accordingly. She received immunoglobulin for persistent thrombocytopenia, after which there was transient improvement. The patient was transferred to our hospital on day 25 after recurrence of fever, watery diarrhea, and a generalized maculopapular rash. On admission, she had tachycardia, tachypnoea, anemia, thrombocytopenia, hypoalbuminemia, and generalized edema...

BACKGROUND: Coronary artery fistulae (CAF) are abnormal connections of a coronary artery to a cardiac chamber or vessel. There is a paucity of data regarding clinical outcomes, especially when detected prenatally. METHODS: This was a multi-center retrospective cohort study of all CAF cases from 2002-2016. Clinical characteristics and outcomes were compared between the prenatal and postnatal cohorts. A scoping literature review of prenatal CAFs was completed. RESULTS: CAFs were diagnosed prenatally in 12 (median 23 (17-36) weeks gestation) and postnatally in 94 (median 2...

OBJECTIVES: Shock is a life-threatening condition in children in low- and middle-income countries (LMIC), with several controversies. This systematic review summarizes the etiology, pathophysiology and mortality of shock in children in LMIC. METHODS: We searched for studies reporting on children with shock in LMIC in PubMed, Embase and through snowballing (up to 1 October 2019). Studies conducted in LMIC that reported on shock in children (1 month-18 years) were included...

BACKGROUND: Neonatal coarctation of the aorta (CoA) is primarily treated by surgical repair. However, under certain high-risk constellations, initial stent angioplasty may be considered followed by surgical repair. We report our experience with this staged approach. Methods: All patients undergoing surgical CoA repair following prior stenting at our institution between January 2011 and December 2019 were included in this retrospective analysis. The patients were classified to be at high risk because of cardiogenic shock, associated complex cardiac malformations, neonatal infection, necrotizing enterocolitis, and extracardiac conditions, respectively...

Coarctation of the aorta (Coa) is a potentially life threatening diagnosis. It occurs in 0.3 per 1000 live births and accounts for 6-8% of all infants with congenital heart defects. Neonates with severe Coa may be completely asymptomatic at birth, as the ductus arteriosus can provide flow to the lower body. Those who are not diagnosed prenatally may be diagnosed only after constriction of the ductus arteriosus, when they present in cardiogenic shock. This group has a higher risk for mortality and morbidity relative to those diagnosed prenatally...