Diagnosis of Neonatal Congenital Heart Disease: A Combination of Heart Murmur, SpO2 Abnormality, Tachypnea, and Extracardiac Malformations

Congenital heart disease (CHD) is one of the commonest congenital malformations that are mostly asymptomatic at birth, which challenges the diagnosis of neonatal CHD. An early accurate prenatal diagnosis will give parents a choice, as well as the opportunity to plan the delivery and improve the postnatal outcome. The purpose of the study is to evaluate the value of heart murmurs, SpO2 abnormalities, tachypnea, and extracardiac malformations in screening neonatal CHD. All 4500 newborns in the obstetrics department of our hospital from January 2019 to January 2020 are selected as study subjects. Newborns were grouped according with the presence of heart murmurs, tachypnea, transdermal SpO 2 < 95 % , and extracardiac malformations alone or in combination (≥3). Patients with murmur, tachypnea, and abnormal SpO2 were assigned into group A, those with murmur, tachypnea, and extracardiac malformations into group B, those with murmurs, SpO2, and extracardiac malformations into group C, those with SpO2, tachypnea, and extracardiac malformations into group D, and those with all four into group E. The color echocardiography identified 65 children with CHD (1.4%) among the included 4,500 newborns. When murmur, tachypnea, abnormal SpO2, and extracardiac malformation were independently used to diagnose CHD, the sensitivity ranged from 30.68% to 51.26%, with specificity ranging from 47.36% to 82.65% and Youden’s index (YI) ranging from 0.13 to 0.36. When murmur, tachypnea, abnormal SpO2, extracardiac malformation were together used to diagnose CHD, 91.23% sensitivity, 95.26 specificity, and 0.91 YI were observed. In conclusion, a combination of four indicators, murmur, tachypnea, abnormal SpO2, and extracardiac malformation yielded good performance in diagnosing neonatal CHD.

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Pulmonary arterial hypertension associated with congenital heart disease in children: clinical characterisation, outcomes and changes in demographics over time

European Heart Journal ◽

10.1093/eurheartj/ehab724.1857 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

A Constantine ◽

K Dimopoulos ◽

R Condliffe ◽

P Clift ◽

G Chaplin ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Free Survival ◽

Paediatric Cohort ◽

Group A ◽

Pulmonary Arterial ◽

Group B ◽

Group D ◽

Over Time

Abstract Background/Introduction Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) in children and is associated with significant morbidity and mortality. The impact of different phenotypes on management and survival remains unclear. Purpose To examine the clinical features, management and outcomes of paediatric PAH-CHD patients based on the 20-year experience of the UK National Paediatric Pulmonary Hypertension (PH) Service. Methods Consecutive PAH-CHD patients entering the service between January 2001 and January 2021 were included and classified into: Eisenmenger syndrome (ES, group A), PAH related to a significant systemic-pulmonary shunt (group B), PAH with small or co-incidental CHD (group C) and PAH following defect repair (group D). Incident patients (without pre-existing PAH) were included in survival analysis. Results Of the overall PH paediatric cohort of 1104 patients, 819 (74.2%) had co-existing CHD and 354 (32.1%) patients received a diagnosis of PAH-CHD: 57.1% female, median [IQR] 4.6 [1.7–10.9] years. Group D PAH-CHD was the commonest subgroup, accounting for 36%, while the least frequent subtype was group C (14%). Group A and group B PAH-CHD represented 26% and 24%, respectively. Down syndrome was present in over one third (122, 34.5%) of PAH-CHD patients and was more commonly associated with ES (p=0.02). PAH therapy was started in 79.9% of PAH-CHD patients. At the end of follow-up, patients with group C PAH-CHD were more likely to be on combination therapy than any other group (64.6% vs. 28.4%, p<0.0001). Prostanoid therapy was used in a minority (11%) of patients. The subgroup distribution of PAH-CHD at diagnosis changed from the early (2000–2005) to late (2015–2020) period (Figure 1). The proportion of ES patients decreased from 43.4% to 14.6% of PAH-CHD (p<0.0001). The proportion of group B PAH-CHD patients increased (9.4% vs. 33.3%, p<0.0001), with the majority (59.3%) deemed “operable” on specialist assessment. There was a trend for an increase in repaired PAH-CHD patients between the early and late era (31.1% vs. 43.8%, p=0.09). Transplant-free survival in PAH-CHD was 90.9% (95% CI: 87.8–94%) at 1 year, 77.9% (95% CI: 73.1–83.1%) at 5 years, and 74.9% (95% CI: 69.6–80.7%) at 10 years (Figure 2). Group C PAH-CHD had a lower transplant-free survival than the other 3 groups (HR 2.54, 95% CI: 1.51–4.28, p=0.0005). There was no difference in outcome between group A and group D PAH-CHD (HR 1.19, 95% CI: 0.62–2.28, p=0.6). Conclusions Repaired PAH-CHD, not ES, was the most common subtype in this large paediatric cohort. Over time, there were fewer ES patients and more “operable” patients with left-right shunts, suggesting an improvement in early diagnosis and management. Despite widespread use of PAH therapy, PAH-CHD remains a life-limiting disease with the poorest outcomes in PAH with co-incidental CHD. FUNDunding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Dr Constantine received a personal PhD fellowship grant (CHAMPION PhD Fellowship) Figure 1 Figure 2

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COMPARISON OF INTRANASAL DEXMEDETOMIDINE V/S MIDAZOLAM AS A PREMEDICATION IN CHIDREN WITH CONGENITAL HEART DISEASE UNDERGOING CARDIAC SURGERY

International Journal of Advanced Research ◽

10.21474/ijar01/12360 ◽

2021 ◽

Vol 9 (01) ◽

pp. 814-822

Author(s):

Sanjay Kumar Morwal ◽

Ajit Singh ◽

C.K. Vyas ◽

Neelu Sharma

Keyword(s):

Congenital Heart Disease ◽

Cardiac Surgery ◽

Heart Disease ◽

Congenital Heart ◽

Arterial Blood ◽

Sedative Effects ◽

Group A ◽

Child Separation ◽

Group B ◽

Intranasal Midazolam

Background: The intranasal route is a reliable way to administer preanaesthetics and sedatives to children. The aim of present study was to compare the anxiolytic and sedative effects of intranasal dexemedetomidine and midazolam as a premedication in children with congenital heart disease undergoing cardiac surgery.. Patients and Methods: Fifty chidrens 2-12 years of age group of either sex with congenital heart disease undergoing cardiac surgery were randomly allocated into two groups. Group A-(n=25) - received intranasal dexmedetomidine as premedication.(0.1 Ã‚Âµg/kg diluted in 2ml NS ) Group B- (n=25)- received intranasal midazolam as premedication. (0.2 mg/kg diluted in 2ml NS) Heart rate, mean arterial blood pressure and oxygen saturation were monitored upto30 minute after drug administration. The sedation score, anxiety score and child separation score were recorded until the child taken to the OR. The postoperative agitation score was also observed. Results and Conclusion: Premedication with intranasal dexmedetomidine attained significant and satisfactory sedation with better parental separation and lower anxiety levels without any adverse effects as compared with intranasal midazolam in children with congenital heart disease undergoing cardiac surgery.

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Efficacy of citrulline supplementation to decrease the risk of pulmonary hypertension after congenital heart disease surgery. A local experience.

Revista de la Facultad de Ciencias Médicas de Córdoba ◽

10.31053/1853.0605.v77.n4.27936 ◽

2020 ◽

Vol 77 (4) ◽

pp. 249-253

Author(s):

Silene Silvera Ruiz ◽

Carola L Grosso ◽

Margot Tablada ◽

Marcelo Cabrera ◽

Raquel Dodelson de Kremer ◽

...

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Congenital Heart ◽

Pediatric Patients ◽

Reference Level ◽

Plasma Citrulline ◽

Post Surgery ◽

Group A ◽

Group B

Pulmonary hypertension (PH) is a major cause of morbi-mortality among patients with congenital heart disease (CHD) and also a potentially severe complication after surgical repair. Oral citrulline, a precursor to NO synthesis, is safe and efficacious for decreasing the risk of postoperative PH. Objective: The aim of the present study was to investigate in pediatric patients the changes of plasma citrulline, arginine, homocysteine and nitric oxide (NO) metabolites and pulmonary artery pressures (PAP) pre-post cardiac surgery in order to describe our population status with regard to the risk of pulmonary hypertension and look for potential biomarkers for early detection and treatment. Main results/Discussion: 16 Argentine pediatric patients with CHD undergoing cardiopulmonary bypass were randomized in two groups: (A) with and (B) without perioperative citrulline supplementation. We found that plasma citrulline median levels before surgery were lower in both groups respect to referential values, probably due to the poor nutritional status of our patients; only group A surpassed post-surgery the minimum recommended level to avoid PH. Furthermore, none of the patients in group A showed mean PAP higher than 20 mmHg, whereas in group B, 67% of the measurements were ≥ than the reference level. Conclusions: We reaffirm that citrulline supplementation it is effective in reducing postoperative pulmonary hypertension and biomarkers could evidence patient status as a translational medicine application.

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Heart Murmurs. If You Allow for Mistakes Costs Are Even Higher!

PEDIATRICS ◽

10.1542/peds.92.2.304b ◽

1993 ◽

Vol 92 (2) ◽

pp. 304-304

Author(s):

WARREN H. TOEWS

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Carcinoembryonic antigen levels are increased with pulmonary output in pulmonary hypertension due to congenital heart disease

Journal of International Medical Research ◽

10.1177/0300060520964378 ◽

2020 ◽

Vol 48 (11) ◽

pp. 030006052096437

Author(s):

Yang Zi-yang ◽

Zhao Kaixun ◽

Luo Dongling ◽

Yin Zhou ◽

Zhou Chengbin ◽

...

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Carcinoembryonic Antigen ◽

Congenital Heart ◽

Pulmonary Artery Hypertension ◽

Group A ◽

Serum Cea ◽

Pulmonary Arterial ◽

Group B

Objective Pulmonary artery hypertension (PAH) is a severe complication of congenital heart disease (CHD). Monitoring of pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) is essential during follow-up. This retrospective study aimed to examine carcinoembryonic antigen (CEA) as an additional marker for evaluation by investigating the correlation between CEA levels and hemodynamics in CHD-PAH. Methods Seventy-six patients with CHD-PAH (mean PAP [mPAP] >25 mmHg and PVR >3 Wood units, group A), 71 patients with CHD and pulmonary hypertension (CHD-PH, mPAP >25 mmHg and PVR ≤3 Wood units, group B), and 102 patients with CHD without PH (mPAP ≤25 mmHg, group C) were enrolled. Serum CEA levels and the relationships between CEA levels and hemodynamic data were assessed. Results Mean serum CEA levels were 1.99±1.61, 2.44±1.82, and 1.58±1.07 ng/mL, mPAP was 58.66±20.21, 30.2±4.83, and 17.31±4.51 mmHg, and PVR was 10.12±7.01, 2.19±0.56, and 2.2±1.1 Wood units in groups A, B, and C, respectively. Mean pulmonary output (PO) was 7.24±3.07, 15.79±5.49, 10.18±4.72 L/minute, respectively. CEA levels were positively correlated with PO and negatively correlated with PVR in all of the patients. Conclusion CEA levels are increased with PO and decreased with PVR in CHD-PH.

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Delayed Onset of Alcuronium Effect in Children with Cyanotic Congenital Heart Disease

Anaesthesia and Intensive Care ◽

10.1177/0310057x9302100211 ◽

1993 ◽

Vol 21 (2) ◽

pp. 197-200 ◽

Cited By ~ 2

Author(s):

J. P. Keneally ◽

P. H. Goonetilleke ◽

I. M. Ramzan

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Plasma Concentrations ◽

Cyanotic Congenital Heart Disease ◽

Disease Group ◽

Normal Children ◽

Significant Difference ◽

Group A ◽

Group B

The effect of alcuronium dichloride (0.3 mg/kg) was studied in seven normal children (Group A), nine with acyanotic congenital heart disease (Group B) and eight with cyanotic disease (Group C). The onset of action was recorded using an integrated electromyograph and blood samples taken for later estimation of plasma concentrations of the drug. The mean time (SD) taken to 75% suppression of twitch height was 1.3(0.8), 1.7(1.0) and 3.8(2.8) minutes, respectively, in each of the three groups. This was significantly slower in Group C compared with both other groups (P<0.05). While six of the Group A patients and seven from Group B reached 95 % paralysis within ten minutes, only two of the cyanosed children achieved this level of relaxation. However, if times to 95% relaxation were extrapolated, there was no significant difference between the groups at 4.5(3.9), 5.8(5.7) and 10.9(6.5) minutes respectively. There was a weak but statistically significant relationship between haematocrit and time to 75% blockade. Maximum twitch depression was similar in all three groups with plasma concentrations at this time being 1.6(0.7), 1.8(0.5) and 2.3(1.4) μg/ml respectively. Again, there was no statistically significant difference between these values. These results confirm that the clinical onset of relaxation is delayed in children with cyanotic congenital heart disease, possibly because of delayed distribution of alcuronium.

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Universal implantation of temporary epicardial pacing wires after surgery for congenital heart disease: necessity or luxury?

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezz285 ◽

2019 ◽

Author(s):

Gopinath Perumal ◽

Supreet Prakash Marathe ◽

Kim S Betts ◽

Jessica Suna ◽

Jim Morwood ◽

...

Keyword(s):

Risk Factors ◽

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Primary Outcome ◽

Postoperative Arrhythmia ◽

Propensity Matching ◽

Group A ◽

Epicardial Pacing ◽

Group B

AbstractOBJECTIVESRoutine implantation of temporary epicardial pacing wires after surgery for congenital heart disease (CHD) has recently been questioned. We evaluated the incidence of arrhythmias, arrhythmias causing haemodynamic compromise and the safety of a strategy of selective implantation of pacing wires in our unit.METHODSAll patients who underwent surgery for CHD using cardiopulmonary bypass between September 2015 and December 2016 were retrospectively enrolled in the study (n = 313). Patients were stratified into group A (universal implantation) and group B (selective implantation). Group B received pacing wires only when postoperative rhythm disturbances were anticipated based on the operating surgeon’s judgement. The primary outcome was arrhythmia causing haemodynamic compromise. Outcomes were compared between unmatched and propensity matched groups.RESULTSForty-eight patients experienced an arrhythmia causing haemodynamic compromise (15.3%). Twenty-three patients (7.3%) experienced an arrhythmia causing haemodynamic compromise that required the use of pacing wires for therapeutic purposes (group A n = 13, group B n = 10, P = 0.34). There were no pacing wire related complications in either group. All patients in group A and 90% in group B had pacing wires when needed (P = 0.435). In group A, 89% of patients had pacing wires which were not used compared with 13% in group B (P < 0.001). Results were unchanged when repeated using propensity matching (81 pairs).CONCLUSIONSThe probability of developing a postoperative arrhythmia requiring therapeutic pacing can be predicted using the risk factors identified in our study. The routine implantation of pacing wires after surgery for CHD is not necessary. A measured reduction from universal implantation is safe.

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Prevalence and diagnostic accuracy of heart disease in children with asymptomatic murmurs

Cardiology in the Young ◽

10.1017/s1047951115000396 ◽

2015 ◽

Vol 26 (3) ◽

pp. 446-450 ◽

Cited By ~ 7

Author(s):

Adziri H. Sackey

Keyword(s):

Heart Disease ◽

Diagnostic Accuracy ◽

Clinical Examination ◽

Clinical Assessment ◽

Congenital Heart ◽

Heart Murmur ◽

Paediatric Cardiologist ◽

Heart Murmurs ◽

Cardiology Clinic ◽

Heart Lesions

AbstractThis study was performed to determine the prevalence of CHD among children referred with asymptomatic murmurs and to determine the diagnostic accuracy of the assessment of asymptomatic heart murmurs by general paediatricians. We reviewed the records of children who had been referred by general paediatricians to a cardiology clinic for further evaluation of a heart murmur. The referring paediatricians’ clinical assessment of the murmur was compared with the cardiologist’s echocardiographic diagnosis. A total of 150 children were referred by paediatricians to a paediatric cardiologist for further assessment of a heart murmur. Out of 150 children, 72 had a paediatrician’s diagnosis of innocent murmur; of these 72 patients, two (3%) had heart disease on echocardiography. In all, after echocardiography, a range of congenital heart lesions was found in 28 (19%) of the 150 children. CHD is not rare among children with asymptomatic heart murmurs. In this series of children with asymptomatic murmurs, 19% had heart lesions on echocardiography. Most, but not all, of the children with heart lesions were identified on clinical examination by general paediatricians.

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Respiratory symptoms (COPD Assessment Test and modified Medical Research Council dyspnea scores) and GOLD-ABCD COPD classification: the LASSYC study

Jornal Brasileiro de Pneumologia ◽

10.36416/1806-3756/e20210156 ◽

2021 ◽

pp. e20210156

Author(s):

Maria Montes de Oca1 ◽

Maria Victorina Lopez Varela2 ◽

Ana Maria B. Menezes3 ◽

Fernando C. Wehrmeister3 ◽

Larissa Ramirez4 ◽

...

Keyword(s):

Respiratory Symptoms ◽

Early Morning ◽

Cross Sectional Study ◽

Kappa Statistics ◽

Cross Sectional ◽

Group A ◽

Group B ◽

Group D ◽

Youden’S Index ◽

Morning Symptoms

Objective: To assess the frequency and severity of 24-hour respiratory symptoms according to COPD GOLD-ABCD classification (2017-version), the distribution of the patients with COPD into GOLD categories using mMRC (=2) or CAT (=10) scores, and agreement between these cut-off points. Methods: In this cross-sectional study (LASSYC study), 24-hour day respiratory symptoms were assessed by the Evaluating Respiratory Symptoms in COPD (E-RS) questionnaire, Nighttime Symptoms of COPD Instrument (NiSCI), Early Morning Symptoms of COPD Instrument (EMSCI), CAT and mMRC scores. Results: Among the 734 patients with COPD, 61% were male, age 69.6±8.7 years, FEV1% post-BD 49.1±17.5%, mMRC 1.8±1.0 and CAT 15.3±.8.1. By mMRC 33.7% were group-A, 29.2% group-B, 10.2% group-C and 26.9% group-D. By CAT 22.3% were group-A, 41% group-B, 4.8% group-C and 31.9% group-D. Using the mMRC the severity of E-RS, NiSCI and EMSCI scores increased from group A to D. Using the CAT, the groups B and D had the higher scores. Agreement between mMRC and CAT was 89.5% (Kappa statistics=75.7%). For mMRC score of 2, CAT score of =11 showed the maximum Youden's index (1.34). For mMRC score of 1, CAT score of =9 and =10 showed the maximum Youden's index (1.48). Conclusion: GOLD COPD classification by CAT seems to better discriminate 24-hour symptoms. Results do not support the equivalent use of CAT=10 and mMRC=2 for assessing symptoms.

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Outcome of children referred with heart murmurs referred from general practice to a paediatrician with expertise in cardiology

Cardiology in the Young ◽

10.1017/s104795111400095x ◽

2014 ◽

Vol 25 (1) ◽

pp. 123-127 ◽

Cited By ~ 5

Author(s):

Abha Khushu ◽

Anthony W. Kelsall ◽

Juliet A. Usher-Smith

Keyword(s):

Primary Care ◽

Congenital Heart Disease ◽

Heart Disease ◽

Family History ◽

General Practitioners ◽

Congenital Heart ◽

Hospital Records ◽

Heart Murmurs ◽

History Of ◽

Referral Practice

AbstractBackgroundHeart murmurs are common in children. The majority are detected incidentally, and congenital heart disease is rare. There are no published United Kingdom guidelines for management of children with murmurs in primary care. Common practice is to refer all children for specialist assessment.AimTo review outcomes of children with heart murmurs who were referred by general practitioners to a clinic conducted by a paediatrician with expertise in cardiology.Design and settingA retrospective review of children referred by general practitioners to a paediatrician with expertise in cardiology.MethodsHospital records of all children under 17 years of age who attended the clinic during 2011 were reviewed. We excluded children previously seen by any cardiology service.ResultsThere were 313 new primary care referrals, with complete records available for 310 children. Of them, 186 (60%) were referred with a murmur: 154 for an asymptomatic murmur alone, and 32 for a murmur plus additional symptoms or family history of congenital heart disease. All underwent echocardiography. Of the patients, 38 (20%) had congenital heart disease. Younger children were more likely to have congenital heart disease. There was no difference between rates of congenital heart disease in children with or without symptoms or a family history.ConclusionThe finding that a large proportion of children referred by general practitioners with asymptomatic heart murmurs have congenital heart disease supports current referral practice on clinical grounds.

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