Congestive Heart Failure In Infancy Research Articles

Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a 10-Day-Old Boy: A Case Report

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare anomaly, comprising approximately 0.1% of all congenital heart diseases. Patients suffered congestive heart failure in infancy, and some patients will experience progressive pulmonary vascular disease without surgical repair. In patients of this disease, early surgical intervention is generally advised and has demonstrated a high level of safety and efficacy, yielding excellent outcomes. We report a unique case involving a 10-day-old boy, characterized by the rare anomalous origination of the right pulmonary artery from the ascending aorta. This case is further complicated by the presence of a patent ductus arteriosus (PDA) and a right descending aorta. The ligation of the PDA and reimplantation of the right pulmonary artery (RPA) were successfully performed. The patient exhibited a favorable recovery trajectory postoperatively.

Association of Preoperative Diuretic Use With Early Acute Kidney Injury in Infants With Biventricular Hearts Following Cardiac Surgery

BackgroundDiuretics are used to manage congestive heart failure in infants with congenital heart disease. Adult data indicate that preoperative diuretic use increases the risk of cardiac surgery associated acute kidney injury (CS‐AKI). We have sought to understand if preoperative diuretics in infants increases the risk of CS‐AKI.Methods and ResultsThis is a single‐center retrospective study of infants (1–12 months) who had CS requiring cardiopulmonary bypass between 2013 and 2018. The diagnosis and severity of CS‐AKI was defined according to the Kidney Disease Improving Global Outcomes guidelines. Three hundred patients were included (mean 6 months, SD 2.4, range 1.2–12.9 months). A total of 149 (49.7%) patients were diagnosed with CS‐AKI (stage 1: 80 [54%], stage 2: 57 [38%], stage 3: 12 [8%]). Logistic regression analysis showed preoperative diuretics were not associated with CS‐AKI (odds ratio [OR], 0.79; 95% CI, 0.43–1.44; P=0.45). A diagnosis of tetralogy of Fallot was an independent risk factor for CS‐AKI (OR, 3.49; 95% CI, 1.33–9.1, P=0.01). A diagnosis of tetralogy of Fallot (OR, 3.6; 95% CI, 1.28–10.22; P=0.02) and longer cardiopulmonary bypass (OR, 1.01; 95% CI, 1.0–1.02; P=0.04) time are risk factors for moderate to severe CS‐AKI.ConclusionsPreoperative diuretic use does not contribute to the risk of CS‐AKI in infants early after surgery. A diagnosis of tetralogy of Fallot was the only risk factor for CS‐AKI identified using multivariate analysis in our cohort. Furthermore, a diagnosis of tetralogy of Fallot and longer cardiopulmonary bypass time are risk factors for moderate to severe CS‐AKI.

Interaction between pulmonary vasculature and the patent ductus arteriosus in very premature infants.

The benefits of closing the ductus arteriosus in very preterm infants have not been convincingly shown in numerous clinical trials. Because a large untreated ductus arteriosus can cause death from congestive heart failure in infants born at term, we need to explain why this might not occur in premature infants born at <28 weeks' gestation. Based on information in the literature, I have commented on the possible relationship between the pulmonary vasculature and the shunt through the patent ductus arteriosus. Many of these infants have bronchopulmonary dysplasia, in which animal and human studies have shown a reduced number of capillaries and small pulmonary arteries as well as reduction in vascular endothelial growth factor (VEGF) and platelet endothelial cell adhesion molecule-1 (PECAM-1). Both of these import angiogenic factors. Some who do not have bronchopulmonary dysplasia may have a restricted pulmonary vascular bed. The increased pulmonary vascular resistance in very premature infants may restrict pulmonary blood flow even if the ductus is large, thus reducing the urgency for ductus closure.

Comparison Between Balloon Angioplasty and Surgical treatment for Native Coarctation of the Aorta in Infants

Coarctation of the aorta (CoA) is a discrete stenosis of the proximal thoracic aorta. The common clinical pattern is congestive heart failure in infancy. Treatment methods include balloon angioplasty (BA) and surgical repair in this age group. Percutaneous balloon angioplasty is a less invasive method for the repair of discrete coarctation but remains controversial as a primary treatment strategy for a native coarctation.&#x0D; This study aimed to compare the effectiveness and outcome of balloon angioplasty and surgical repair in infants with coarctation of the aorta younger than one year old.&#x0D; Methods. Between January 2011 and December 2017, 59 patients with native aortic coarctation were treated in National Amosov Institute of Cardiovascular Surgery. This retrospective study evaluated the results of the two methods in patients younger than one year old with the diagnosis of coarctation of the aorta. Group 1 included 18 patients who accepted balloon dilatation for discrete coarctation. Group 2 included 41 patients who underwent surgical resection with end-to-end anastomosis. Patients with complex cardiac anomalies were not included in this study.&#x0D; Results. Immediate results revealed no significant difference in the effectiveness of the two methods (p value =0.06). While the rate of recurrent coarctation was significantly lower in the surgery group [1 (2,4%) vs. 13 (72,2%), p value =0.0017]. The mean hospital stay was 6.4±3.6 days in the balloon angioplasty group and 21.5±8.7 days in the surgery group, which constitutes a significant statistical difference (p value &lt;0.05). Aneurysm formation was not encountered.&#x0D; Conclusion. Both surgical repair and balloon angioplasty for native coarctation of the aorta in infants were effective and beneficial. These data suggest that balloon angioplasty can be acceptable alternative to surgical treatment but due to the high level of recoarctation this method may be considered in critically ill infants with congestive heart failure as a primary palliative procedure. The limitations of this study were its retrospective, nonrandomized nature, and smaller case numbers.

Safety of Enalapril in Infants Admitted to the Neonatal Intensive Care Unit.

Enalapril is used to treat hypertension and congestive heart failure in infants. However, enalapril is not labeled for neonates, and safety data in infants are sparse. To evaluate the safety of enalapril in young infants, we conducted a retrospective cohort study of infants who were exposed to enalapril in the first 120days of life and were cared for in 348 neonatal intensive care units from 1997 to 2012. We determined the proportion of exposed infants who developed adverse events, including death, hypotension requiring pressors, hyperkalemia, and elevated serum creatinine. Using multivariable logistic regression, we examined risk factors for adverse events, including postnatal age at first exposure, exposure duration, gestational age group, small for gestational age status, race, sex, 5-min Apgar score, and inborn status. Of a cohort of 887,910 infants, 662 infants (0.07%) were exposed to enalapril. Among exposed infants, 142 infants (21%) suffered an adverse event. The most common adverse event was hyperkalemia (13%), followed by elevated serum creatinine (5%), hypotension (4%), and death (0.5%). Significant risk factors for adverse events included postnatal age <30days at first exposure and longer exposure duration. This study is the largest to date examining the safety of enalapril in young term and preterm infants without significant structural cardiac disease.

Cardiac beriberi presenting as severe PAH with impending congestive heart failure in infants of poor socioeconomic background

Cardiac beriberi presenting as severe PAH with impending congestive heart failure in infants of poor socioeconomic background

Chronic congestive heart failure in infancy and childhood: new aspects of diagnosis and treatment.

Congestive heart failure (CHF) is the inability of the heart to meet the metabolic demands of the body. As a disease of the advanced age with a frequency of 1-2% of the population it is rare in infancy and childhood. The incidence ranges from 2.95 (in all US children's hospitals) to 23.2 (University's Childrens Hospital Essen) on 1 000 discharges. Among the diagnostic procedures echocardiography is the primary modality of imaging. Tei-index and tissue-Doppler are more sensitive parameters for LV-dysfunction. BNP/NT-proBNP are age-dependent and can guide the long-term therapy. Treatment in childhood does not differ basically from that in adulthood, recommended by several guidelines, but data regarding the various substances - outlined in detail - are very limited. Here a big work has to be done in future!

Successful Surgical Outcome of Coarctation of Aorta in an adult male

Coarctation of the aorta is a relatively common defect that accounts for 5-8% of all congenital heart defects. Coarctation of the aorta may occur as an isolated defect or in association with various other lesions, most commonly patent ductus arteriosus, bicuspid aortic valve and ventricular septal defect. The diagnosis of coarctation of the aorta may be missed or delayed unless there is a high index of suspicion and the presentation may be congestive heart failure in infant or hypertension in older children and adult. A case of coarctation of the aorta associated with patent ductus arteriosus having features of headache, exertional dyspnea and uncontrolled hypertension is being presented here. PTFE patch aortoplasty and ligation with transfixation of PDA was done. Post-operative period was uneventful and patient was discharged on 7th post-operative day. DOI: http://dx.doi.org/10.3329/uhj.v8i1.11671 University Heart Journal Vol. 8, No. 1, January 2012

Percutaneous closure of an atrial septal defect in an infant with shone's syndrome

Rarely is closure of the atrial septal defect indicated in infants. Yet, an atrial septal defect in the presence of congenital mitral stenosis may cause significant congestive heart failure in infancy. We present the case of an infant with Shone's syndrome, left ventricular hypoplasia, and a large ostium secundum atrial septal defect who developed pulmonary overcirculation following repair of coarctation of the aorta. The infant underwent cardiac catheterization, hemodynamic assessment, and successful percutaneous closure of the atrial septal defect. The patient improved dramatically following the intervention. Follow-up echocardiograms have demonstrated growth of mitral valve annulus diameter and left ventricular dimensions. Atrial septal defect closure in this small infant was associated with immediate improved clinical status, and improved growth of the left heart structures.

Presentation of Congenital Heart Disease in the Neonate and Young Infant

1. Michael Silberbach, MD* 2. David Hannon, MD† 1. *Editorial Board 2. †East Carolina University, Greenville, NC After completing this article, readers should be able to: 1. Describe the general principles of the cardiac examination of the newborn. 2. Name the four presentations of congenital heart disease in the newborn period. 3. Recognize the innocent heart murmurs that occur during infancy. 4. Identify the signs and symptoms of congestive heart failure in infants. 5. Describe the pathophysiology of the more common cyanotic heart defects. Significant congenital heart disease (CHD) may be diagnosed at virtually any age. Some conditions always are discovered in neonates; others rarely are identified during infancy. Prenatal ultrasonography may detect CHD before birth, but it is essential for primary care practitioners to assess any newborn who is suspected of having heart disease at the time of birth. This review considers CHDs that present during the newborn period or early infancy. The profound hemodynamic transitions that occur at the time of birth make the clinical presentation of heart disease a “moving target.” Although echocardiography often reveals the anatomic details of a given lesion, an understanding of underlying pathophysiologic principles can provide the clinician with the tools to anticipate and treat problems as they arise. Visual inspection of the infant may reveal cyanosis, but it is equally important to recognize dysmorphic features. Congenital heart lesions associated with some specific syndromes are summarized in Table 1, and the incidence of most common cardiac malformations is presented in Table 2. Assessment of the precordial activity is as important as cardiac auscultation. Palpation of the intensity and timing of the upper and lower extremity pulses as well as measurement of blood pressure in all four extremities are critical in the assessment of left heart obstructive disease. Auscultation should focus on the heart sounds as well as any murmurs. | Etiologic Syndrome | Frequency of Cardiac Anomalies† | Distinguishing Features | |:----------------------------------------------:| ------------------------------- | ---------------------------------------------------------- | -------------------------------------------------------------------------------------- | | All (%) | Distinctive or Most Common | | Autosomal Dominant | | | | | Adams-Oliver syndrome | 20 | Left-sided obstruction (eg, COA, parachute MVP), TOF | Scalp cutis aplasia, terminal transverse limb defects | | Alagille syndrome | 95 | (P)PS, TOF/TOF with PA, ASD, VSD | Bile duct paucity, chronic cholestasis, butterfly vertebrae, posterior embryotoxon | | Char syndrome | 60 | PDA | Anomalies on fifth finger, supernumerary nipple | | Cornelia de Lange syndrome | 25 | VSD, ASD, PS, TOF | Upper limb deficiency, GI anomalies | | Holt-Oram syndrome | 80 | ASD± other CVM, VSD, TA, TOF, PAPVC, conduction defect | Upper limb malformations | | Neurofibromatosis | 2 | PSV, ASV, COA, HCM | Cafe au lait macules, optic glioma, scoliosis, pseudarthrosis, neurofibromas | | Noonan syndrome | 85 | PSV, ASD, AVSD partial, COA, HCM | Short, webbed neck; pectus deformity; cryptorchidism | | Rubinstein-Taybi syndrome | 35 | PDA, ASD, VSD, left-sided obstruction (eg, COA, HLHS) | Broad thumbs and great toes | | Williams syndrome | 60 | SVAS, PS, other left-sided obstructions (eg, ASV, MS, COA) | Hypercalcemia, hypodontia, hypoplastic nails | | Autosomal Recessive | | | | | Ellis-van Creveld syndrome | 60 | AVSD, common atrium, ASD primum | Short limbs, polydactyly, hypoplastic nails, dental anomalies | | Fryns syndrome | 50 | ASD, VSD, conotruncal | Diaphragmatic hernia, distal digital hypoplasia | | Keutel syndrome | 70 | (P)PS | Short digits, mixed hearing loss, cartilage calcification | | Smith-Lemli-Opitz syndrome | 45 | ASD, VSD, complete AVSD, TAPVC | Two- to three-toe syndactyly, cleft palate, lung anomalies, genital anomalies | | X-linked Recessive | | | | | Simpson-Golabi-Behmel syndrome | 25 | ASD; VSD; rare, variable cardiomyopathy | Macrosomia, cleft palate, supernumerary nipples, hernias, hypospadias, poly/syndactyly | | Suspected Gene Etiology | | | | | Cardio-facio-cutaneous syndrome | 75 | ASD, HCM | Sparse, curly hair; low, rotated ears; hyperkeratosis | | Hall-Hittner syndrome (CHARGE association) | 80 | Conotruncal/arch, assorted CVMs | Coloboma, choanal atresia, genital anomalies, ear anomalies | | Costello syndrome | 60 | MVP, AV, thickening HCM, arrhythmia (atrial tachycardia) | Skin/joint laxity, fine/curly hair, deep palm creases, ulnar deviation, papillomata | | PHACES syndrome | 100 | COA; IAA, A right; double, cervical aortic arch | Posterior fossa malformations, hemangiomas, eye anomalies | | Ritscher-Schinzel syndrome (3C) | 100 | TOF, DORV, AVSD | Posterior fossa malformations, cleft palate, coloboma | * ASD=atrial septal defect, ASV=aortic stenosis, valvar, AV=atrioventricular valve, AVSD=atrioventricular septal defect, COA=coarctation, CVM=cardiovascular malformations, …

Kardiologische Notfälle im Kindesalter

There are relatively few situations where cardiac emergencies can occur in a child. A special focus is directed to the cyanotic newborn. In these cases a very specific treatment plan is necessary which is limited to a few dedicated centres. The importance lies in the timely diagnosis of a cyanotic heart defect and the rapid and safe transport from the referring hospital. An outline is given on the causes and treatment of congestive heart failure in infancy. Management guidelines are given for the most frequent types of cardiac arrhythmias and the few occasions, where an arrhythmia can lead to a real emergency, mostly in infants. To complete the list of the most frequent cardiac emergencies in pediatrics, pericardial effusion/tamponade is mentioned as well as the occurrence of cyanotic spells typically in the baby with Fallot's tetralogy.

532 Congestive heart failure in infancy and childhood: an epidemiological study

532 Congestive heart failure in infancy and childhood: an epidemiological study

Usefulness of the mitral valve Z score in predicting the need to close moderate- to large-sized atrial septal defects in infants with aortic coarctation

Left ventricular anatomic substrates predisposing the development of congestive heart failure in infants with atrial left-to-right shunting after aortic coarctation repair were characterized. Mitral valve, but not aortic or left ventricular, hypoplasia correlated with the development of heart failure and the need for atrial defect closure shortly after coarctation repair.

Congestive Heart Failure in Infants and Children.

Congestive heart failure (CHF) refers to a clinical state of systemic and pulmonary congestion resulting from inability of the heart to pump as much blood as required for the adequate metabolism of the body. The clinical picture of CHF results from a combination of “relatively low output” and compensatory responses to increase it. Excellent reviews on CHF in infants and children are available [1, 2]. This article provides core information on selected aspects of CHF. Broadly, heart failure results either from an excessive volume or pressure overload on normal myocardium (left to right shunts, aortic stenosis), or from primary myocardial abnormality (myocarditis, cardiomyopathy). Arrhythmias, pericardial diseases and combination of various factors can also result in CHF. The resultant decrease in cardiac output triggers a host of physiological responses aimed at restoring perfusion of the vital organs [3]. Important among these are renal retention of fluid, renin-angiotensin mediated vasoconstriction and sympathetic overactivity. Excessive fluid retention increases the cardiac output by increasing the end diastolic volume (preload), but also results in symptoms of pulmonary and systemic congestion. Vasoconstriction (increase in afterload) tends to maintain flow to vital organs, but it is disproportionately elevated in patients with CHF and increases myocardial work. Similarly, sympathetic over-activity results in increase in contractility, which also increases myocardial requirements. An understanding of the interplay of the four principal determinants of cardiac output – preload, afterload, contractility and heart rate is essential in optimising the therapy of CHF. It is clinically useful to consider CHF in different age groups separately.

Propranolol treatment of congestive heart failure in infants with congenital heart disease: The CHF-PRO-INFANT Trial

Aim: Infants with congenital heart disease and left-to-right shunts may develop significant clinical symptoms of congestive heart failure in spite of therapy with digoxin and diuretics. We investigated the effects of β-blockade in infants with severe heart failure. Methods and results: We performed a prospective, randomized, open monocenter trial in infants treated with digoxin and diuretics ( n=10) in comparison to 10 infants receiving additional β-blocker therapy. After 17 days on average β-blocker treated infants (propranolol:1,6 mg/kg/day) improved significantly with respect to Ross heart failure score (3.3±2.3 vs. 8.3±1.9, P=0.002), lower renin levels (338±236 vs. 704±490 μU/l, P=0.008) and lower mean heart rates in Holter ECG (118±10 vs. 142±11 beats/min, P<0.001). While digoxin and diuretic treated infants had unchanged mean heart rate (149±8 vs. 148±10 beats/min), less decrease of symptoms (Ross Score: 8.5±1.7 vs. 6.8±2.3, P=0.02) but a significant increase of renin levels (139±102 vs. 938±607 μU/l, P=0.001). Conclusion: Additional propranolol treatment but not digoxin and diuretics alone can effectively reduce clinical symptoms of heart failure in infants with congenital heart disease, who suffer from increased neurohormonal activation.

Beta-Blocker Therapy of Severe Congestive Heart Failure in Infants With Left to Right Shunts

We report on the clinical and neurohumoral effects of adding low-dose propranolol to conventional therapy with digoxin and diuretics in 6 infants with severe congestive heart failure due to large left-to-right shunts. A significant decrease in heart failure scores and a decrease of the highly activated renin-angiotensin-1 aldosterone system by approximately 70% strongly suggests a beneficial effect of this new therapeutic approach.

Malalignment-type ventricular septal defect in double-chambered right ventricle

Double-chambered right ventricle (DCRV) is commonly associated with ventricular septal defect (VSD). In this study, an assessment was made of the relevance of a malalignment-type VSD to hemodynamic and morphologic features in DCRV. During an 8.5-year period, 53 patients with DCRV were enrolled after study with echocardiography, catheterization, and angiography. Patients were divided into 2 groups: group I included 40 patients, aged 3.7 +/- 3.2 years, with a malalignment-type VSD; group II consisted of 13 patients, aged 8.6 +/- 2.7 years, without a malalignment-type VSD. History of congestive heart failure in infancy was present in 21 group I and 2 group II patients (53% vs 15%, respectively, p <0.05). The mean pulmonary-to-systemic flow ratio was significantly higher in group I than in group II (1.89 +/- 0.74 vs 1.14 +/- 0.21, respectively, p <0.05). The mean pressure gradient across the right ventricular outflow tract was lower in group I than in group II (41 +/- 16 vs, 73 +/- 33 mm Hg, respectively, p <0.05). Among 42 patients who had a series of echocardiograms recorded, progression of pressure gradient was evident in 35: 28 in group I and 7 in group II. A subaortic ridge was present exclusively in 29 group I patients (73%). Prolapse of the aortic valve was present in 26 (49%): 20 group I (50%) and 6 group II (46%) patients. Aneurysm formation of the septal defect was found in 17 (43%) and 7 (54%) group I and II patients, respectively. It can be concluded that a history of congestive heart failure was more common in DCRV patients with a malalignment-type VSD. Malalignment-type VSD is significantly associated with a larger pulmonary-to-systemic flow ratio and subaortic ridge.

Traitement de l'insuffisance cardiaque aiguë du nourrisson

The treatment of congestive heart failure in infants must be etiological and symptomatic. Vasodilators, inotropic agents and diuretics are the main therapeutics used in the symptomatic treatment. Guidelines for the emergency treatment according to the main etiologies are presented.

Increased plasma norepinephrine levels in infants and children with congestive heart failure.

To characterize the changes in circulating catecholamine levels for children with varying degrees of congestive heart failure (CHF), plasma norepinephrine (NE) and epinephrine (E) levels were measured, using high performance liquid chromatography, in 94 noncyanotic congenital heart disease patients. Plasma NE levels in 43 patients with CHF were significantly higher than those in the 51 patients without CHF (704 +/- 68 pg/ml vs 274 +/- 68 pg/ml, p < 0.001). Compared with the controls, the NE levels in the mild, moderate and severe CHF subgroups increased by 2.0, 2.6 and 3.7 fold, respectively. The E concentration did not significantly vary among the groups. The plasma NE levels correlated directly with the degree of left-to-right shunt (r = 0.46, p < 0.001) and pulmonary systolic pressure (r = 0.67, p < 0.001). Thus, a highly significant association was found between the level of plasma NE and severity of CHF symptoms. A follow-up study of 15 of the 30 CHF patients after surgery disclosed a significant decline in plasma NE (p < 0.001). The results of this study clearly demonstrate that plasma NE levels parallel the presence and severity of CHF in infants and children.

CURRENT MANAGEMENT OF CONGESTIVE HEART FAILURE IN INFANTS AND CHILDREN

Although the general goals of therapy for the medical and nursing management of CHF in children have not changed in the last decade, advances have been made in understanding the unique characteristics of the neonatal heart and tailoring therapy to best support cardiac function (Table 6). In addition, strides have been made in manipulating the loading conditions of the ventricle to enhance cardiac output, which has fostered the development of new therapeutic agents and more aggressive treatment of these patients with improved outcomes. In situations in which the infant is unresponsive to therapy, surgical intervention is now done at an earlier age with good results. When surgery is not an option and the patient continues to deteriorate despite maximum medical management, mechanical support can be instituted as a bridge to transplantation.