ADAPTATIONAL GROWTH OF DUCTUS ARTERIOSUS AND AORTIC ISTHMUS IN CRITICAL CONGENITAL HEART DISEASE WITH OBSTRUCTED SYSTEMIC CIRCULATION

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.

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Low-dose prostaglandin E1 is safe and effective for critical congenital heart disease: is it time to revisit the dosing guidelines?

Cardiology in the Young ◽

10.1017/s1047951120003297 ◽

2020 ◽

pp. 1-8

Author(s):

Daniel Vari ◽

Wendi Xiao ◽

Shashank Behere ◽

Ellen Spurrier ◽

Takeshi Tsuda ◽

...

Keyword(s):

Mechanical Ventilation ◽

Congenital Heart Disease ◽

Heart Disease ◽

Respiratory Depression ◽

Congenital Heart ◽

Prostaglandin E1 ◽

Low Dose ◽

Systemic Circulation ◽

Critical Congenital Heart Disease ◽

Starting Dose

Abstract Introduction: Prostaglandin E1 is used to maintain ductal patency in critical congenital heart disease (CHD). The standard starting dose of prostaglandin E1 is 0.05 µg/kg/minute. Lower doses are frequently used, but the efficacy and safety of a low-dose regimen of prostaglandin E1 has not been established. Methods: We investigated neonates with critical CHD who were started on prostaglandin E1 at 0.01 µg/kg/minute. We reviewed 154 consecutive patients who were separated into three anatomical groups: obstruction to systemic circulation, obstruction to pulmonary circulation, and inadequate mixing (d-transposition of the great arteries). Treatment failure rates and two commonly reported side effects, respiratory depression and seizure, were studied. Results: A total of 26 patients (17%) required a dose increase in prostaglandin E1. Patients with pulmonary obstruction were more likely to require higher doses than patients with systemic obstruction (15/49, 31% versus 9/88, 10%, p = 0.003). Twenty-eight per cent of patients developed respiratory depression and 8% of patients needed mechanical ventilation. Prematurity (<37 week gestation) was the primary risk factor for respiratory depression. No patient required dose escalation or tracheal intubation while on transport. No patient had a seizure attributed to prostaglandin E1. Conclusions: Prostaglandin E1 at an initial and maintenance dose of 0.01 µg/kg/minute was sufficient to maintain ductal patency in 83% of our cohort. The incidence of respiratory depression requiring mechanical ventilation was low and was mostly seen in premature infants. Starting low-dose prostaglandin E1 at 0.01 µg/kg/minute is a safe and effective therapy for critical CHD.

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Lipo-PGE1 treatment of the neonate with critical congenital heart disease and ductus-arteriosus dependent circulation

Advanced Drug Delivery Reviews ◽

10.1016/0169-409x(95)00121-m ◽

1996 ◽

Vol 20 (2-3) ◽

pp. 177-180 ◽

Cited By ~ 10

Author(s):

Kazuo Momma

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Ductus Arteriosus ◽

Critical Congenital Heart Disease

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Critical congenital heart disease screening with a pulse oximetry in neonates

Journal of Perinatal Medicine ◽

10.1515/jpm-2017-0006 ◽

2018 ◽

Vol 46 (2) ◽

pp. 203-207 ◽

Cited By ~ 1

Author(s):

Şahin Hamilçıkan ◽

Emrah Can

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulse Oximetry ◽

Congenital Heart ◽

Septal Defect ◽

Ductus Arteriosus ◽

Newborn Infants ◽

Added Value ◽

Critical Congenital Heart Disease ◽

Pulse Oximetry Screening

AbstractObjective:To compare the results of pulse oximetry screening for critical congenital heart disease (CCHD) in newborn infants performed at <24 h and >24 h following.Method:Measurements were taken for each group at <24 h and >24 h following birth. Echocardiography was performed if the SpO2 readings remained abnormal results.Results:A total of 4518 newborns were included in this prospective descriptive study. Of these, 2484 (60.3%) were delivered vaginally and 1685 (39.7%) by cesarean section. Median time points of the screening were 25.4 (25.3–25.5) vs. 17.3 (12.2–22.4) hours after birth. In 4109 infants screened 24 h after birth, the mean pre- and postductal oxygen saturations (SpO2) were 96.5±1.99 and 97.7±1.98, while 127 infants screened within 24 h of mean preductal and postductal SpO2 were 91.33±2.64 and 94.0±4.44. No CCHD was detected during the study period. Pulse oximetry screening was false positive for CCHD in 9 of 4109 infants (0.02%); of these, six infants were referred to pediatric cardiology and three cases were diagnosed as other significant, non-cardiac pathology. There were two cases with AVSD (atrioventricular septal defect, three cases with ventricular septal defect (VSD), and one case with patent ductus arteriosus (PDA).Conclusions:Saturation values are different between <24-h and >24-h neonates in pulse oximetry screening. The screening in this study identified infants with other important pathologies, this forms an added value as an assessment tool for newborn infants.

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

The Journal of Critical Care Medicine ◽

10.1515/jccm-2016-0031 ◽

2016 ◽

Vol 2 (4) ◽

pp. 185-191 ◽

Cited By ~ 3

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.

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Critical Congenital Heart Disease in Newborn: Early Detection, Diagnosis, and Management

BioScientia Medicina ◽

10.32539/bsm.v5i1.180 ◽

2020 ◽

Vol 5 (1) ◽

pp. 107-116

Author(s):

Herick Alvenus Willim ◽

Cristianto ◽

Alice Inda Supit

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Early Detection ◽

Congenital Heart ◽

Care Center ◽

Ductus Arteriosus ◽

Tertiary Care ◽

Critical Congenital Heart Disease ◽

Diagnosis And Management ◽

Discharge From Hospital

Critical congenital heart disease (CHD) is a type of CHD that requires early intervention in the first year of life to survive. Morbidity and mortality increases significantly if newborns with critical CHD experience delay in the initial diagnosis and management. The infants may develop cyanosis, systemic hypoperfusion, or respiratory distress as the main manifestations of critical CHD. Pulse oximetry screening for early detection of critical CHD must be performed in newborns after 24 hours of age or before discharge from hospital. Generally, infants with critical CHD require patency of the ductus arteriosus with infusion of prostaglandin to maintain pulmonary or systemic blood flow. After initial management, the infants must be immediately referred to tertiary care center for definitive intervention. Keywords: congenital heart disease, duct-dependent circulation, ductus arteriosus, prostaglandin

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Implementation of Critical Congenital Heart Disease Screening in Minnesota

PEDIATRICS ◽

10.1542/peds.2013-0803d ◽

2013 ◽

Vol 132 (3) ◽

pp. X14-X14

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Disease Screening ◽

Critical Congenital Heart Disease

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Pulse Oximetry Screening Has Not Changed Timing of Diagnosis or Mortality of Critical Congenital Heart Disease

Pediatric Cardiology ◽

10.1007/s00246-020-02330-1 ◽

2020 ◽

Vol 41 (5) ◽

pp. 899-904 ◽

Cited By ~ 1

Author(s):

Matthew J. Campbell ◽

William O. Quarshie ◽

Jennifer Faerber ◽

David J. Goldberg ◽

Christopher E. Mascio ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulse Oximetry ◽

Congenital Heart ◽

Critical Congenital Heart Disease ◽

Pulse Oximetry Screening

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

American Journal of Perinatology ◽

10.1055/s-0039-1685445 ◽

2019 ◽

Vol 37 (02) ◽

pp. 158-165 ◽

Cited By ~ 3

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.

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