Congenital Heart Disease

2016 ◽  
Author(s):  
Zachary Pittsenbarger ◽  
Emily Roben
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Congenital Heart ◽  
Ductus Arteriosus ◽  
Flow Patterns ◽  
Prostaglandin E ◽  
Office Visits ◽  
Outflow Obstruction ◽  
Blood Flow Patterns

Congenital heart disease (CHD) is common, affecting approximately 8 in 1000 live-born children, and encompasses a broad range of diagnoses and presentations.  CHDs can include inborn derangements in almost any aspect of the hearts structure of function, but the most common type of congenital heart disease are structural lesions of the heart that affect the normal pattern of blood flow.  These structural lesions can present with varied symptoms and physical exam signs that are rooted in their underlying blood flow patterns often lead to one of four groups of diseases based on blood flow patterns.  Overcirculation, systemic outflow obstruction, systolic failure, and cyanosis are the four groups used as descriptive classifications of structural CHD.  CHD findings can be present prenatally, in the newborn nursery, and well child office visits, but very often present to the EDs without any cardiac history when the lesion progresses to a point of crisis when the cardiac output is no longer meeting the body’s perfusion demands.  Early presentations of CHD frequently are related to closure of the ductus arteriosus and may benefit from early treatment with prostaglandin E.  Lab tests, radiology studies, and exam findings may be suggestive of certain types of lesions, but the gold standard to determine the type of CHD is an echocardiogram.  Once the diagnosis of CHD is suspected, consultation with a pediatric cardiologist is highly recommended to arrange the timely evaluation of the child and prompt initiation of therapies if needed to mitigate the disease progression.                         Key words: Congenital heart disease, overcirculation, systemic outflow obstruction, systolic failure, and cyanosis, ductus arteriosus

Three-dimensional and four-dimensional flow assessment in congenital heart disease

Heart ◽  
2019 ◽  
Vol 106 (6) ◽  
pp. 421-426 ◽  
Author(s):  
Evangeline Warmerdam ◽  
Gregor J Krings ◽  
Tim Leiner ◽  
Heynric B Grotenhuis
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Vascular Function ◽  
Congenital Heart ◽  
Surgical Techniques ◽  
Flow Patterns ◽  
Congenital Defects ◽  
4D Flow ◽  
Blood Flow Patterns

Congenital heart disease (CHD) is the most common form of congenital defects, with an incidence of 8 per 1000 births. Due to major advances in diagnostics, perioperative care and surgical techniques, the survival rate of patients with CHD has improved dramatically. Conversely, although 70%–95% of infants with CHD survive into adulthood, the rate of long-term morbidity, which often requires (repeat) intervention, has increased. Recently, the role of altered haemodynamics in cardiac development and CHD has become a subject of interest. Patients with CHD often have abnormal blood flow patterns, either due to the primary cardiac defect or as a consequence of the surgical intervention(s). Research suggests that these abnormal blood flow patterns may contribute to diminished cardiac and vascular function. Serial assessment of haemodynamic parameters in patients with CHD may allow for improved understanding of the often complex haemodynamics in these patients and thereby potentially guide the timing and nature of interventions with the aim of preventing progression of cardiovascular deterioration. In this article we will discuss two novel non-invasive four-dimensional (4D) techniques to evaluate cardiovascular haemodynamics: 4D-flow cardiac magnetic resonance and computational fluid dynamics. This review focuses on the additional value of these two modalities in the evaluation of patients with CHD with abnormal flow patterns, who could benefit from advanced haemodynamic evaluation: patients with coarctation of the aorta, bicuspid aortic valve, tetralogy of Fallot and patients after Fontan palliation.

Prolonged Prostaglandin E1 Infusion in an Infant With Cyanotic Congenital Heart Disease

PEDIATRICS ◽  
1978 ◽  
Vol 61 (4) ◽  
pp. 534-536
Author(s):  
Alan B. Lewis ◽  
Paul R. Lurie
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Gestational Age ◽  
Small For Gestational Age ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Prostaglandin E1 ◽  
Ductus Arteriosus ◽  
Cyanotic Congenital Heart Disease

A small-for-gestational-age premature infant with severe tetralogy of Fallot was treated with prostaglandin E1 to dialate the ductus arteriosus and increase pulmonary blood flow. The infusion was continued for 29 days without complication at which time surgery was performed.

scholarly journals Congenital Heart Disease Requiring Maintenance of Ductus Arteriosus in Critically Ill Newborns Admitted at A Tertiary Neonatal Intensive Care Unit

2016 ◽  
Vol 2 (4) ◽  
pp. 185-191 ◽  
Author(s):  
Manuela Cucerea ◽  
Marta Simon ◽  
Elena Moldovan ◽  
Marcela Ungureanu ◽  
Raluca Marian ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Side Effects ◽  
Pulmonary Circulation ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Ductus Arteriosus ◽  
Prostaglandin E ◽  
Critical Congenital Heart Disease ◽  
The Mean

AbstractIntroduction: Congenital heart diseases (CHD) have been reported to be responsible for 30 to 50% of infant mortality caused by congenital disabilities. In critical cases, survival of newborns with CHD depends on the patency of the ductus arteriosus (PDA), for maintaining the systemic or pulmonary circulation. The aim of the study was to assess the efficacy and side effects of PGE (prostaglandin E) administration in newborns with critical congenital heart disease requiring maintenance of the ductus arteriosus.Material and method: All clinical and paraclinical data of 66 infants admitted to one referral tertiary level academic center and treated with Alprostadil were analyzed. Patients were divided into three groups: Group 1: PDA dependent pulmonary circulation (n=11) Group 2: PDA dependent systemic circulation (n=31) Group 3: PDA depending mixed circulation (n=24)Results: The mean age of starting PGE1 treatment was 2.06 days, 1.91 (+/−1.44) days for PDA depending pulmonary flow, 2.39 (+/−1.62) days for PDA depending systemic flow and 1.71 (+/1.12) for PDA depending mixing circulation. PEG1 initiation was commenced 48 hours after admission for 72%, between 48-72 hours for 6%, and after 72 to 120 hours for 21% of newborns detected with PDA dependent circulation. Before PEG1 initiation the mean initial SpO2 was 77.89 (+/− 9.2)% and mean initial oxygen pressure (PaO2) was 26.96(+/−6.45) mmHg. At the point when stable wide open PDA was achieved their mean SpO2increased to 89.73 (+/−8.4)%, and PaO2 rose to 49 (+/−7.2) mmHg. During PGE1 treatment, eleven infants (16.7%) had apnea attacks, five children (7.5%) had convulsions, 33 (50%) had fever, 47 (71.2%) had leukocytosis, 52 (78.8%) had edema, 25.8% had gastrointestinal intolerance, 45.5% had hypokalemia, and 63.6% had irritability.Conclusions: For those infants with severe cyanosis or shock caused by PDA dependent heart lesions, the initiation and maintenance of PGE1 infusion is imperative. The side effects of this beneficial therapy were transient and treatable.

A retrospective analysis of a pediatric tele-echocardiography service to treat, triage, and reduce trans-Pacific transport

2017 ◽  
Vol 24 (3) ◽  
pp. 224-229 ◽  
Author(s):  
Christopher A Rouse ◽  
Brandon T Woods ◽  
C Becket Mahnke
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Ductus Arteriosus ◽  
Tertiary Care ◽  
The United States ◽  
Critical Congenital Heart Disease ◽  
Medical Centers ◽  
Prompt Diagnosis ◽  
Patent Ductus

Introduction Tele-echocardiography can ensure prompt diagnosis and prevent the unnecessary transport of infants without critical congenital heart disease, particularly at isolated locations lacking access to tertiary care medical centers. Methods We retrospectively reviewed all infants who underwent tele-echocardiography at a remote 16-bed level IIIB NICU from June 2005 to March 2014. Tele-echocardiograms were completed by cardiac sonographers in Okinawa, Japan, and transmitted asynchronously for review by pediatric cardiologists in Hawaii. Results During the study period 100 infants received 192 tele-echocardiograms: 46% of infants had tele-echocardiograms completed for suspected patent ductus arteriosus, 28% for suspected congenital heart disease, 12% for possible congenital heart disease in the setting of likely pulmonary hypertension, and 10% for possible congenital heart disease in the setting of other congenital anomalies. Of these, 17 patients were aeromedically evacuated for cardiac reasons; 12 patients were transported to Hawaii, while five patients with complex heart disease were transported directly to the United States mainland for interventional cardiac capabilities not available in Hawaii. Discussion This study demonstrates the use of tele-echocardiography to guide treatment, reduce long and potentially risky trans-Pacific transports, and triage transports to destination centers with the most appropriate cardiac capabilities.

Cerebrovascular Blood Flow Dynamic Changes in Fetuses with Congenital Heart Disease

2009 ◽  
Vol 25 (1) ◽  
pp. 167-172 ◽  
Author(s):  
Lv Guorong ◽  
Li Shaohui ◽  
Jin Peng ◽  
Lin Huitong ◽  
Li Boyi ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Congenital Heart ◽  
Flow Dynamic ◽  
Dynamic Changes ◽  
Blood Flow Dynamic

Oxygen Saturation and Perfusion Index-Based Enhanced Critical Congenital Heart Disease Screening

2019 ◽  
Vol 37 (02) ◽  
pp. 158-165 ◽  
Author(s):  
Heather Siefkes ◽  
Laura Kair ◽  
Daniel J. Tancredi ◽  
Brian Vasquez ◽  
Lorena Garcia ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Oxygen Saturation ◽  
Congenital Heart ◽  
Perfusion Index ◽  
Outflow Obstruction ◽  
Critical Congenital Heart Disease ◽  
Healthy Infants ◽  
Optimal Screening ◽  
To Come

Objective To determine if addition of perfusion index (PIx) to oxygen saturation (SpO2) screening improves detection of critical congenital heart disease (CCHD) with systemic outflow obstruction. Study Design We determined screening thresholds for PIx and applied these to a cohort of newborns with and without congenital heart disease (CHD). Results A total of 123 normal and 21 CHD newborns (including five with critical systemic outflow obstruction) were enrolled. Four of these five critical systemic obstruction subjects passed SpO2-based screen. Four out of these five subjects failed PIx-based screen. The sensitivity for detection of systemic obstruction CCHD when compared with healthy infants increased from 20% (95% confidence interval [CI]: 1–72%) with SpO2 screening alone to 80% (95% CI: 28–100%) with combined SpO2-PIx screen. However, 2.44% of normal infants failed PIx screen. Conclusion Addition of PIx to SpO2 screening may detect additional cases of CCHD and further research is necessary to come up with optimal screening thresholds.

Tracheobronchial Compression in Acyanotic Congenital Heart Disease

1983 ◽  
Vol 92 (4) ◽  
pp. 387-390 ◽  
Author(s):  
Norman T. Berlinger ◽  
John Foker ◽  
Charles Long ◽  
Russell V. Lucas
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Main Bronchus ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Tracheobronchial Tree ◽  
Middle Lobe ◽  
Lobe Bronchus ◽  
Acyanotic Congenital Heart Disease

Children with acyanotic congenital heart disease frequently develop respiratory difficulties such as atelectasis, pneumonia, or infantile lobar emphysema. In some cases, the cause of the respiratory difficulty is compression of the tracheobronchial tree by hypertensive dilated pulmonary arteries, since this type of heart disease frequently demonstrates large left-to-right intracardiac shunts. Sites of predilection for compression include the left main bronchus, the left upper lobe bronchus, the junction of the right bronchus intermedius and right middle lobe bronchus, and the left side of the distal trachea. Cardiac anomalies which predispose to this type of compression include ventricular septal defect, patent ductus arteriosus, interruption of the aortic arch, and tetralogy of Fallot. Pulmonary arteriopexy may relieve the tracheobronchial compression.

Dilatable Pulmonary Artery Banding Palliation in Congenital Heart Disease

2021 ◽  
Vol 12 (2) ◽  
pp. 213-219
Author(s):  
R. Allen Ligon ◽  
Larry A. Latson ◽  
Mark M. Ruzmetov ◽  
Kak-Chen Chan ◽  
Immanuel I. Turner ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Balloon Dilation ◽  
Retrospective Chart Review ◽  
Pulmonary Artery Banding ◽  
Surgical Removal

Background: Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients. Methods: Retrospective chart review of adjustable PAB—outline anatomical variants palliated and patient outcomes. Results: Sixteen patients underwent dilatable PAB—median age 52 days (range 4-215) and weight 3.12 kg (1.65-5.8). Seven (44%) of the patients were premature, 11 (69%) had ventricular septal defect(s) with pulmonary over-circulation, four (25%) atrioventricular septal defects, and four (25%) single ventricle physiology. Subsequent to the index procedure: five patients have undergone intracardiac complete repair, six patients remain well palliated with no additional intervention, and four single ventricles await their next palliation. One patient died from necrotizing enterocolitis (unrelated to PAB) and one patient required a pericardiocentesis postoperatively. Five patients underwent TCBD of the PAB without complication—Two had one TCBD, two had two TCBD, and another had three TCBD. The median change in saturation was 14% (complete range 6-22) and PAB diameter 1.7 mm (complete range 1.1-5.2). Median time from PAB to most recent outpatient follow-up was 868 days (interquartile range 190-1,079). Conclusions: Our institution has standardized a PAB technique that allows for transcatheter incremental increases in pulmonary blood flow over time. This methodology has proven safe and effective enough to supplant other institutional techniques of limiting pulmonary blood flow in most patients—allowing for interval growth or even serving as the definitive palliation.

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