COR PULMONALE IN INFANCY SIMULATING CONGENITAL HEART DISEASE

PEDIATRICS ◽  
1954 ◽  
Vol 14 (6) ◽  
pp. 587-601
Author(s):  
IAN MAXWELL ◽  
REGINALD WILSON
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Clinical Picture ◽  
Congenital Heart ◽  
Cor Pulmonale ◽  
The Other ◽  
Vascular Lesions ◽  
Cardiac Anomalies ◽  
Congenital Cardiac Anomalies

1. Two cases of pulmonary arteriolar obstruction associated with hypertension of the lesser circulation have been described. Both cases were considered during life to be suffering from some form of congenital heart disease. 2. The pathogenesis of the pulmonary vascular lesions has been discussed. In one case, the lesion appears to have been a congenital dysplasia of arterioles throughout both lungs. In the other necrotizing arteriolitis was present in one lung which was also atelectatic. It was absent in the other well aerated lung though vascular lesions associated with pulmonary hypertension were present bilaterally. 3. The clinical picture of these patients has been described. It is suggested that this is sufficiently distinctive to permit clinical recognition and differentiation from congenital cardiac anomalies.

scholarly journals Parameters associated with outcome in pediatric patients with congenital heart disease and pulmonary hypertension subjected to combined vasodilator and surgical treatments

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401983788 ◽  
Author(s):  
Ana Maria Thomaz ◽  
Luiz J. Kajita ◽  
Vera D. Aiello ◽  
Leína Zorzanelli ◽  
Filomena Regina B.G. Galas ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Vascular Resistance ◽  
Congenital Heart ◽  
Postoperative Outcome ◽  
Vascular Lesions ◽  
Cardiac Lesions ◽  
Medial Hypertrophy ◽  
Surgical Treatments

Management of pediatric pulmonary hypertension associated with congenital heart disease (PHT-CHD) is challenging. Some patients have persistently elevated pulmonary artery pressure (PAP) after cardiac surgery, an undesired condition that is difficult to predict. We investigated the value of clinical, hemodynamic, and histopathological data in predicting the outcome in a prospective cohort. Patients with PHT-CHD received sildenafil orally pre- and postoperatively for six months and then were subjected to a catheter study. Thirty-three patients were enrolled (age range = 4.6–37.0 months). Pulmonary vascular resistance (PVR) was 4.9 (range = 3.9–7.2) Wood units × m2 (median with IQR). Twenty-two patients had a ≥ 20% decrease in PVR and pulmonary-to-systemic vascular resistance ratio (PVR/SVR) in response to inhaled nitric oxide (NO). The response was directly related to the degree of medial hypertrophy of pulmonary arterioles ( P < 0.05) (morphometric analysis, intraoperative lung biopsy). Subsequently, five of the non-responders had a ≥ 30% increase in pulmonary blood flow in response to sildenafil (3.0 [2.0–4.0] mg/kg/day). Six months after surgery, PAP and PVR were significantly lower ( P < 0.001 vs. baseline), even in seven patients with Heath-Edwards grade III/IV pulmonary vascular lesions ( P = 0.018), but still abnormal in 12 individuals (>25 mmHg and >3.0 U × m2, respectively). A preoperative PVR/SVR of ≥24% during NO inhalation and a wall thickness of arteries accompanying respiratory bronchioli of ≥4.7 (Z score) were identified, respectively, as risk and protection factors for abnormal postoperative hemodynamics (hazard ratio [95% CI] = 1.09 [1.01–1.18], P = 0.036; and 0.69 [0.49–0.98], P = 0.040, respectively). Thus, in PHT-CHD patients receiving oral sildenafil pre- and post-surgical repair of cardiac lesions, mid-term postoperative outcome is predictable to some extent.

Trazodone-Associated Syncope in a Patient with Congenital Cardiac Anomalies

1998 ◽  
Vol 14 (2) ◽  
pp. 78-81
Author(s):  
Vinod S Bhatara ◽  
Sami Awadallah
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Noonan Syndrome ◽  
Congenital Heart ◽  
Cardiac Defects ◽  
Congenital Cardiac Defects ◽  
Cardiac Anomalies ◽  
Case Summary ◽  
Congenital Cardiac Anomalies

Objective: To report a case of trazodone-associated syncope in a 19-year-old woman with congenital cardiac anomalies and to review risk factors and mechanisms involved in this case. Case Summary: A 19-year-old woman diagnosed with Noonan syndrome with congenital cardiac anomalies tolerated sertraline, but had a syncopal episode when trazodone was used. There was no recurrence of syncope after the trazodone was discontinued. Discussion: The syncopal episode could have resulted from either trazodone or congenital heart disease, but it most likely resulted from a trazodone–congenital heart disease interaction. Trazodone is not cardiotoxic in most patients; our patient was probably vulnerable to the cardiotoxic effects of trazodone due to congenital cardiac defects. Conclusions: Trazodone can precipitate arrhythmias or syncope in susceptible patients with heart disease and should be prescribed cautiously in patients with congenital heart disease. In such cases, it may be safer to use a selective serotonin-reuptake inhibitor.

scholarly journals Congenital Heart Disease and Surgical Outcome in Down Syndrome

2021 ◽  
Author(s):  
Zainab Al-Suhaymi
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Current Knowledge ◽  
Innovative Technology ◽  
Mortality And Morbidity ◽  
Cardiac Anomalies ◽  
Congenital Cardiac Anomalies ◽  
Causes Of Mortality

The prevalence of congenital heart disease has accounted for nearly one-third of all significant congenital anomalies worldwide. The first report about an association between cardiac anomalies and Down Syndrome was in (1876). Ten years after discovering of Down Syndrome and the credit of association between congenital cardiac anomalies and mongolism was suggested in (1894) by Garrod. There many studies performed to identify a correlation between genotype and phenotype in Down Syndrome, little is known about cardiovascular phenotype in Down Syndrome. Congenital heart disease is considered one of the highest causes of mortality and morbidity in Down Syndrome compared to patients with the same lesion of non-down. There is a big debate about surgical management and considered them as risk factors of surgery with precaution and recent technology, Down Syndrome considered as a normal patient in prognosis. This chapter aimed to shed the light on congenital heart disease in Down Syndrome and current knowledge in specific mutations associated with them and how the effect of innovative technology and management to treat them end at the same outcome and sometimes better based on recent research and Scoring System.

Congenital Malformations of the Heart

PEDIATRICS ◽  
1948 ◽  
Vol 1 (2) ◽  
pp. 298-298
Author(s):  
JAMES GOODFRIEND
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Malformations ◽  
Congenital Heart ◽  
Clinical Knowledge ◽  
Cardiac Anomalies ◽  
Congenital Cardiac Anomalies ◽  
Circulatory Dynamics

Seldom has a monograph or text been so eagerly awaited as Dr. Taussig's summation of her observations of congenital malformations of the heart, and rarely has a book so completely justified the pre-publication interest it evoked as does this. Dr. Taussig's investigations of congenital cardiac anomalies have for some years been directed toward increasing clinical knowledge of these conditions and have been related to Dr. Maude Abbott's classical pathologic studies. All the clinical types of congenital heart disease are here discussed in detail. The embryologic aberration is pointed out. With the nature of the anatomic deformity established, the inevitable alterations in circulatory dynamics are then considered and the conclusions in each instance are presented in diagrammatic form.

Publishing patterns and citation performance of manuscripts relating to paediatric cardiology and congenital heart disease: comparison of paediatric and adult cardiology journals

2021 ◽  
pp. 1-5
Author(s):  
Rohit S. Loomba ◽  
Danielle Sheikholeslami ◽  
Aaron Dyson ◽  
Saul Flores ◽  
Enrique Villarreal ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Median Number ◽  
Lower Number ◽  
The Other ◽  
Google Scholar ◽  
H Index ◽  
Paediatric Cardiology ◽  
Number Of Citations

Abstract Background: Manuscripts pertaining to paediatric cardiology and CHD have been published in a variety of different journals. Some of these journals are journals dedicated to paediatric cardiology, while others are focused on adult cardiology. Historically, it has been considered that manuscripts published in journals devoted to adult cardiology have greater citation potential. Our objective was to compare citation performance between manuscripts related to paediatric cardiology and CHD published in paediatric as opposed to adult cardiology journals. Methods: We identified manuscripts related to paediatric cardiology and CHD published in five journals of interest during 2014. Of these journals, two were primarily concerned with adult cardiology, while the other three focused on paediatric cardiology. The number of citations for these identified manuscripts was gathered from Google Scholar. We compared the number of citations (median, mean, and 25th, 75th, 90th, and 95th percentiles), the potential for citation, and the h-index for the identified manuscripts. Results: We identified a total of 828 manuscripts related to paediatric cardiology and congenital heart as published in the 5 journals during 2014. Of these, 783 (95%) were published in journals focused on paediatric cardiology, and the remaining 45 (5%) were published in journals focused on adult cardiology. The median number of citations was 41 in the manuscripts published in the journals focused on adult cardiology, as opposed to 7 in journals focused on paediatric cardiology (p < 0.001). The h-index, however, was greater for the journals dedicated to paediatric cardiology (36 versus 27). Conclusion: Approximately one-twentieth of the work relating to paediatric cardiology and CHD is published in journals that focus predominantly on adult cardiology. The median number of citations is greater when manuscripts concerning paediatric cardiology and CHD are published in these journals focused on adult cardiology. The h-index, however, is higher when the manuscripts are published in journals dedicated to paediatric cardiology. While such publications in journals that focus on adult cardiology tend to generate a greater number of citations than those achieved for works published in specialised paediatric cardiology journals, the potential for citation is no different between the journals. Due to the drastically lower number of manuscripts published in journals dedicated to adult cardiology, however, median performance is different.

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

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