THE TREATMENT OF THE PIERRE ROBIN SYNDROME

PEDIATRICS ◽  
1962 ◽  
Vol 30 (3) ◽  
pp. 450-458
Author(s):  
Marvin H. Goldberg ◽  
Roger H. Eckblom
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Clinical Symptoms ◽  
The Other ◽  
Pierre Robin Syndrome ◽  
Traction Device ◽  
Method Of Production ◽  
Traction System ◽  
Pierre Robin

The diagnosis of the Pierre Robin Syndrome, cleft palate, micrognathia, and golssoptosis is re-emphasized. The clinical symptoms are discussed. The treatments advocated in the literature are described. A simple traction device, employing a horizontal suture through the mid-body of the tongue and an elastic traction system, is explained. Five cases of the syndrome are reported. In one the condition was mild; in the other four the tongue traction device was employed. In one sick infant with a congenital heart disease a tracheotomy had to be performed. Much difficulty was experienced following this, and the operation is only recommended when traction alone does not control the handling of the profusion of mucus and bouts of apnea. A removable acrylic palatine obturator was used as an adjunct to nipple feedings in three patients, with excellent results. The method of production of this "false palate" is described.

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.

Outcomes and Prognostic Factors for Adult Patients With Congenital Heart Disease Undergoing Primary or Reoperative Systemic Atrioventricular Valve Surgery

2017 ◽  
Vol 8 (3) ◽  
pp. 346-353
Author(s):  
Elizabeth H. Stephens ◽  
Jiho Han ◽  
Jonathan Ginns ◽  
Marlon Rosenbaum ◽  
Paul Chai ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Prognostic Factors ◽  
Adverse Events ◽  
Congenital Heart ◽  
Atrioventricular Septal Defect ◽  
The Other ◽  
Mitral Valve Stenosis ◽  
Atrioventricular Valve ◽  
Exact Test

Background: Adults with congenital heart disease (ACHD) undergoing systemic atrioventricular valve (SAVV) surgery are a complex, understudied population. We assessed midterm outcomes and prognostic factors in ACHD undergoing SAVV surgery. Methods: We performed retrospective evaluation of ACHD undergoing SAVV surgery from January 2005 to February 2016: 14 (33%) patients with congenital mitral valve stenosis/regurgitation, 15 (35%) with atrioventricular septal defect (AVSD), and 14 (33%) with congenitally corrected transposition of the great arteries (ccTGA) with systemic tricuspid valve regurgitation. Adverse events were defined as mortality, reoperation on SAVV, and late more-than-moderate (> moderate) SAVV regurgitation. Statistical analysis was performed using Fisher’s exact test and one-way analysis of variance as well as univariate and multivariate risk factor analysis. Results: Fifteen (35%) patients had preoperative systemic ventricular dysfunction, including 13 patients with ccTGA (93%, P < .001). Twenty-three (54%) patients underwent valve repair, 20 (47%) patients underwent replacement, and 20 (47%) patients underwent an associated procedure. Replacement was higher in patients with ccTGA (86%) than the other groups ( P < 0.01). Thirty-seven patients (91% of survivors) were free of significant SAVV regurgitation at last follow-up, with patients with AVSD having greater regurgitation grades compared to the other groups ( P < 0.01). In-hospital mortality, late mortality, late > moderate SAVV regurgitation, and SAVV reoperation rates were 5% (n = 2), 2% (n = 1), 9% (n = 3), and 7% (n = 3), respectively. On multivariate analysis, predischarge SAVV regurgitation grade was the only significant predictor of adverse events (odds ratio = 8.2, 95% confidence interval: 1.1-63.8, P = .045). Conclusion: Overall outcomes in this challenging population are good. The single factor associated with adverse events was predischarge SAVV regurgitation grade.

scholarly journals Gambaran Penyakit Jantung Bawaan di Neonatal Intensive Care Unit RSUP Prof. Dr. R. D. Kandou Manado Periode 2013 - 2017

e-CliniC ◽  
2018 ◽  
Vol 6 (2) ◽  
Author(s):  
Berry R. Manopo ◽  
Erling D. Kaunang ◽  
Adrian Umboh
Keyword(s):  
Intensive Care Unit ◽  
Congenital Heart Disease ◽  
Intensive Care ◽  
Heart Disease ◽  
Neonatal Intensive Care Unit ◽  
Neonatal Intensive Care ◽  
Congenital Heart ◽  
Septal Defect ◽  
Clinical Symptoms ◽  
Shortness Of Breath

Abstract: Congenital heart disease (CHD) is a structural heart defect that results from abnormal embryological heart development, or persistence of some parts of the fetal circulation at birth. Congenital heart disease is divided into two categories, namely non-cyanotic congenital heart disease and cyanotic congenital heart disease. Congenital heart disease is caused by interactions between predisposing exogenous factors and endogenous factors. This study was aimed to obtain the profile of CHD in the Neonatal Intensive Care Unit (NICU) of Prof. Dr. R. D. Kandou Hospital Manado in the period 2013 - 2017. This was a retrospective descriptive study using medical record data of patients suffering from CHD in NICU from 2013 to 2017. The results showed that there were 27 patients suffering from CHD consisting of 24 non-cyanotic CHD patients (88.89%) and 3 cyanotic CHD patients (11.11%), and the highest incidence was Atrial Septal Defect (ASD) as many as 17 babies (62.96%). Congenital heart disease was more common in males as many as 18 babies (66.67%). In this study, the clinical symptoms oftenly found was shortness of breath (48.15%) and the most common diagnosis was pneumonia (48.15%). Conclusion: The most common CHD was non-cyanotic CHD. The most commonly found defect was ASD. Clinical symptoms that often arised was shortness of breath, pneumonia was the most common comorbid diagnosis, and the dominant gender of CHD was male.Keywords: non-cyanotic CHD, cyanotic CHD, atrial septal defect Abstrak: Penyakit jantung bawaan (PJB) merupakan defek jantung struktural yang terjadi akibat perkembangan jantung embriologis yang abnormal, atau persistensi dari beberapa bagian dari sirkulasi fetus saat lahir. Penyakit ini dibagi menjadi dua kategori yaitu penyakit jantung bawaan non sianosis dan yang sianosis. Penyakit jantung bawaan disebabkan oleh interaksi antara predisposisi faktor eksogen dan faktor endogen. Penelitian ini bertujuan untuk mendapatkan gambaran penyakit jantung bawaan di Neonatal Intensive Care Unit (NICU) RSUP Prof. Dr. R. D. Kandou Manado periode 2013-2017. Jenis penelitian ialah deskriptif retrospektif dengan menggunakan data rekam medik pasien yang menyandang penyakit jantung bawaan di NICU periode 2013-2017. Hasil penelitian mendapatkan dari 27 pasien dengan PJB, ditemukan PJB non sianotik berjumlah 24 bayi (88,89%) dan PJB sianotik berjumlah 3 bayi (11,11%) dengan angka kejadian terbanyak pada atrial septal defek (ASD) berjumlah 17 bayi (62,96%). Penyakit jantung bawaan paling banyak terjadi pada bayi yang berjenis kelamin laki-laki yaitu berjumlah 18 bayi (66,67%). Gejala klinis yang sering muncul ialah sesak napas (48,15%) dan diagnosis penyerta terbanyak yaitu pnemonia (48,15%). Simpulan: Penyakit jantung bawaan non sianosis merupakan diagnosis terbanyak, jenis ASD, dengan gejala klinis yang sering muncul yaitu sesak napas. Pneumonia merupakan diagnosis penyerta terbanyak. PJB tersering pada jenis kelamin laki-laki.Kata kunci: PJB sianotik, PJB, non sianotik, atrial septal defek

ONE OF THE EARLIEST AMERICAN CASE REPORTS OF A CHILD WITH CONGENITAL HEART DISEASE (1816)

PEDIATRICS ◽  
1976 ◽  
Vol 57 (1) ◽  
pp. 91-91
Author(s):  
T. E. C.
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Clinical Symptoms ◽  
Case Reports ◽  
Natural State ◽  
Long Time ◽  
Hot Weather ◽  
American Case ◽  
Cold Wind

Clinical descriptions of heart disease in children, congenital or acquired, are extremely rare prior to 1784 when Michael Underwood published his textbook on The Diseases of Children. In a chapter in his book Underwood summed up what had already been discovered about congenital heart disease and for the first time attempted to connect it with clinical symptoms. Dr. Robert Thaxter's case report of a child with a malformed heart is one of the earliest I have found in American medical journals. The subject of this case, a male, was at birth apparently well formed and healthy and remained very well 8 or 10 days. At this time he was attacked with the ordinary symptoms of cold which continued a long time very troublesome. He became very emaciated; which was attributed partly to the above cause, and partly to the mother, whose health was at this time bad. He remained in this emaciated state till he was about a year old at which time he began to grow corpulent and continued so till his death; though his extremities were small compared with his body and head. As soon as he began to move much, he was observed to breathe with difficulty, and his countenance to become livid. Rest generally restored him to a natural state. Cold, especially a cold wind, affected his respiration, sometimes almost producing suffocation, and rendered his surface very livid. Very hot weather had nearly as bad an affect as cold. Eating anything very hot produced the same effect.

Twins and Congenital Heart Disease

1961 ◽  
Vol 10 (04) ◽  
pp. 443-456 ◽  
Author(s):  
Maurice Campbell
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Malformation ◽  
Genetic Predisposition ◽  
Congenital Heart ◽  
Monozygotic Twins ◽  
The Other ◽  
Unpublished Paper ◽  
Dizygotic Twins ◽  
Foetal Circulation

Summary and Conclusions The number of twins in 942 families where the propositus had a congenital malformation of the heart has been studied. The 38 pairs were not significantly more than could be expected by chance: nor were the 21 pairs where one member had a malformation of the heart. In 16 pairs, one was affected and the other normal; and in 5 pairs, one was affected and the other unknown. In 2 pairs, both were unknown because of early deaths; and in 15, both members of the pair were normal. In no pair were both members known to be affected. There is clear evidence from this series and from the series of Uchida and Rowe (1957) and of Lamy et al. (1957) that where one member of a pair of monozygotic twins has a congenital malformation of the heart, the other is generally normal. It is the exception rather than the rule for both members to be affected, though several examples of such pairs have been reported. There is some evidence from these three series that monozygotic twins are more liable to have a malformation of the heart than dizygotic twins and their sibs. It follows from these two findings, that the immediate cause of the malformation of the heart must be environmental, though probably there is some genetic predisposition. A disturbance of the foetal circulation to the affected twin because of a single placenta and chorion seems to be the most likely environmental cause. I should like to thank Professor Gedda of Rome and Professor Polani for their helpful suggestions and Dr. I. E. Evans for letting me see his unpublished paper on the evidence for monozygosity in some of the pairs of twins included in this paper.

Malformations Complexes des Membres Supérieurs Associées à une Cardiopathie Congénitale. À propos de six observations

1965 ◽  
Vol 14 (2) ◽  
pp. 132-163 ◽  
Author(s):  
I. Emerit ◽  
J. de Grouchy ◽  
M. Laval-Jeantet ◽  
P. Corone ◽  
P. Vernant
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Atrial Septal Defect ◽  
High Frequency ◽  
Congenital Heart ◽  
Septal Defect ◽  
The Other ◽  
Upper Limbs ◽  
Cardiopathie Congénitale ◽  
Heart Malformations

SUMMARYThe authors report on six patients exhibiting a congenital heart disease and malformations of the upper limbs. In two of them this association is similar to that described by Holt and Oram in 1960: atrial septal defect, heart arrythmia and abnormal thumbs. The other four patients have the same skeletal anomalies but different heart malformations. Dermatoglyphic studies revealed a high frequency of a distal position of the axial triradius and of an equivalent of a simian crease. The karyotypes are all normal. The etiology is discussed.

Health-related quality of life in adolescents with congenital heart disease

2014 ◽  
Vol 25 (3) ◽  
pp. 526-532 ◽  
Author(s):  
Juliana Bertoletti ◽  
Giovana C. Marx ◽  
Sérgio P. Hattge ◽  
Lúcia C. Pellanda
Keyword(s):  
Quality Of Life ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Clinical Symptoms ◽  
Well Being ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related

AbstractPurpose: This study aims to evaluate the health-related quality of life of adolescents with congenital heart disease, describing health-related quality of life according to the diagnosis and severity of congenital heart disease, identified by variables such as the presence of symptoms, surgical interventions, use of medication, and residual lesion. Methods: A cross-sectional study was conducted on 203 adolescents with congenital heart disease attended at the Pediatric Cardiology ambulatory of a reference hospital in Brazil. The Brazilian version of the questionnaire KIDSCREEN-27 was used for the assessment of health-related quality of life. Information related to the congenital heart disease diagnosis and clinical variables were collected from the medical records of the patients. Results: There was no statistically significant difference between acyanotic and cyanotic groups, as well as for the different diagnosis of congenital heart disease. A trend for better health-related quality of life in the dimension of Psychological Well-Being (p=0.054) was found in the groups with surgical intervention and use of medication. Adolescents that referred a good general health presented significantly better results in all dimensions of health-related quality of life, except for Autonomy and Parent Relation, than those who presented clinical symptoms (p<0.05). Conclusions: Type of congenital heart disease and initial diagnosis did not seem to affect the perception of health-related quality of life, corroborating findings of several studies. Surgical interventions and the use of medication can improve previous clinical status, and therefore it seems to be beneficial in terms of Psychological Well-Being. The presence of clinical symptoms was the variable that caused the largest impact on the perception of health-related quality of life, possibly because of the impairment they bring to the daily lives of these patients.

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