Arachnodactyly Heart

PEDIATRICS ◽  
1959 ◽  
Vol 24 (4) ◽  
pp. 522-522
Author(s):  
S. G.
Keyword(s):  
Myocardial Hypertrophy ◽  
Coarctation Of The Aorta ◽  
Hereditary Disease ◽  
Pathologic Finding ◽  
Cardiovascular Pathology ◽  
Cardiac Malformations ◽  
Septal Defects ◽  
Aneurysm Formation ◽  
Cardiac Decompensation ◽  
Myocardial Pathology

Cardiovascular lesions are known to be frequently associated with arachnodactyly. The most common pathologic finding is cystic medionecrosis, particularly affecting the aorta, which often gives rise to aneurysm formation. Congenital cardiac malformations have also been described in patients with arachnodactyly; the more common lesions encountered are coarctation of the aorta and septal defects. From his experience at the Gronigen Clinic, Netherlands, the author reports another, but less frequently observed, finding of myocardial hypertrophy and fibrosis, in the absence of other cardiovascular pathology; this lesion leads eventually to cardiac decompensation and demise. The term "arachnodactyly heart" is proposed to designate the primary myocardial pathology in this hereditary disease.

Increased Prevalence of Ventricular Septal Defect: Epidemic or Improved Diagnosis

PEDIATRICS ◽  
1989 ◽  
Vol 83 (2) ◽  
pp. 200-203
Author(s):  
Gerard R. Martin ◽  
Lowell W. Perry ◽  
Charlotte Ferencz
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Coarctation Of The Aorta ◽  
Cardiovascular Malformations ◽  
Ventricular Septal Defects ◽  
Live Births ◽  
Septal Defects ◽  
Clinical Diagnoses ◽  
Control Study

The Baltimore-Washington Infant Study is an ongoing case-control study of congenital cardiovascular malformations in infants in whom the clinical diagnoses have been confirmed by echocardiography, catheterization, surgery, or autopsy. An increase in the prevalence of ventricular septal defects was detected in 1,494 infants with congenital cardiovascular malformations between 1981 and 1984. The prevalence of congenital cardiovascular malformations increased from 3.6 to 4.5 per 1,000 live births (P<.025) and the prevalence of ventricular septal defect increased from 1.0 to 1.6 per 1,000 live births (P< .001). The increase in ventricular septal defects accounted for the total increase in congenital cardiovascular malformations. The prevalence of isolated ventricular septal defect increased from 0.67 to 1.17 per 1,000 live births (P<.001). The prevalence of ventricular septal defect with associated coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and pulmonic stenosis did not change. The prevalence of ventricular septal defect diagnosed by catheterization, surgery, and autopsy did not change; however, defects diagnosed by echocardiography increased from 0.30 to 0.70 per 1,000 live births (P<.001). It is concluded that the reported increase in prevalence of ventricular septal defect is due to improved detection of small, isolated ventricular septal defects and that there is no evidence of an "epidemic."

scholarly journals Coarctation of the aorta and post-stenotic aneurysm formation.

Heart ◽  
1990 ◽  
Vol 64 (5) ◽  
pp. 332-333 ◽  
Author(s):  
I M Mitchell ◽  
J C Pollock
Keyword(s):  
Coarctation Of The Aorta ◽  
Aneurysm Formation

scholarly journals P1465 Ventricular septal defect and pulmonary artery aneurysm; How does it look by three dimensional echocardiography

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
H S A Abdelgawad ◽  
M Abdelnabi ◽  
A Almaghrabi ◽  
M Shehata ◽  
M A Abdelhay
Keyword(s):  
Shear Stress ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Maximum Diameter ◽  
Surgical Closure ◽  
Septal Defects ◽  
Aneurysm Formation

Abstract Introduction Pulmonary artery (PA) aneurysms (PAAs) are rare and infrequently diagnosed . The upper limit of the main PA diameter (29 mm) was defined as a PAA. Congenital causes have been recognized as the major reason for PAA formation. More than 50% of all cases were associated with congenital heart disease. The 3 most frequent congenital heart defects associated with a PAA are, in decreasing order, persistent ductus arteriosus (PDA), ventricular septal defects (VSD) , and atrial septal defects (ASD).En face visualization of the whole circumference of the VSD and its relation to the surrounding structures can help in better understanding of the shear stress that promote PAA formation. Case report A 35 year old female patient with no previous cardiac history .She presented to our medical facility complaining of exertional dyspnea since 2 years . On clinical examination, she had harsh pansystolic murmur heard over left parasternal area . 2D Transthoracic Echocardiography revealed an aneurysmally dilated pulmonary artery (40mm in maximum diameter) and a wide VSD. 3D transthoracic and transesophageal echocardiography showed a wide subarterial VSD which is immediately located below the pulmonary valve. Therefore , that increased flow caused by left-to-right shunt caused by the intimate location of the VSD to the pulmonary valve results in increased hemodynamic shear stress on the vessel walls and therefore promotes aneurysm formation .Surgical closure of the VSD was done successfully with no residual defects. Conclusion PAAs seldom occur, are rarely diagnosed, and do not present with distinct symptoms. With the help of 3D echocardiography , direct visualization of the surrounding septal defects helps better understanding of mechanism of the aneurysm formation. Abstract P1465 Figure.

Congenital heart disease in 37,294 births in Tunisia: birth prevalence and mortality rate

2013 ◽  
Vol 24 (5) ◽  
pp. 866-871 ◽  
Author(s):  
Dorra Abid ◽  
Anis Elloumi ◽  
Leila Abid ◽  
Souad Mallek ◽  
Hajer Aloulou ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Coarctation Of The Aorta ◽  
Ventricular Septal Defects ◽  
Birth Prevalence ◽  
Septal Defects

AbstractAim: To investigate the previously unknown birth incidence, treatment, and mortality of children with congenital heart disease in Tunisia. Methods: We undertook a retrospective review of medical records of all patients who were born in 2010 and 2011, and were diagnosed in Sfax (Tunisia) with congenital heart defect. Results: Among 37,294 births, 255 children were detected to have congenital heart disease, yielding a birth incidence of 6.8 per 1000. The most frequently occurring conditions were ventricular septal defects (31%), ostium secundum atrial septal defects (12.9%), and pulmonary valve abnormalities (12%). Coarctation of the aorta, tetralogy of Fallot, univentricular physiology, pulmonary atresia with ventricular septal defect, and transposition of the great arteries were found in 4.3%, 6.2%, 3.4%, 2.7%, and 2.7%, respectively. During the follow-up of 1 year, 23% of the children died. About three-quarters of those deaths happened before surgery. Conclusion: The present study is in line with the general estimates in the world. It has revealed a high case of mortality among the patients awaiting corrective surgery. These children need more facilities.

scholarly journals Transverse arch hypoplasia predisposes to aneurysm formation at the repair site after patch angioplasty for coarctation of the aorta

1995 ◽  
Vol 26 (2) ◽  
pp. 521-527 ◽  
Author(s):  
Jan Bogaert ◽  
Marc Gewillig ◽  
Frank Rademakers ◽  
Hilde Bosmans ◽  
Johny Verschakelen ◽  
...  
Keyword(s):  
Coarctation Of The Aorta ◽  
Repair Site ◽  
Aneurysm Formation ◽  
Transverse Arch ◽  
Patch Angioplasty

Cardiovascular malformations in Omani Arab children with Down's syndrome

2007 ◽  
Vol 17 (2) ◽  
pp. 166-171 ◽  
Author(s):  
Olufemi Jaiyesimi ◽  
Vijaymani Baichoo
Keyword(s):  
Septal Defect ◽  
Down's Syndrome ◽  
Coarctation Of The Aorta ◽  
Down’S Syndrome ◽  
Atrioventricular Septal Defect ◽  
Cardiovascular Malformations ◽  
Arab Children ◽  
Septal Defects ◽  
Appropriate Care ◽  
Echocardiographic Evaluation

Background and aim: Reports from several countries suggest that the prevalence and spectrum of the congenital cardiac malformations seen in the setting of Down's syndrome vary in different ethnic groups and countries, and at different periods in the same country. Data on Arab children are lacking. Our study aimed to fill that void by ascertaining the pattern in Omani Arabs. Methods: Prospective clinical and echocardiographic evaluation of consecutively recruited Omani children with Down's syndrome. Results: We studied 110 children, aged from one day to 11 years, with a median of 2 months, detecting 76 cardiovascular malformations in 63 (57%) of them. Atrioventricular septal defect, the most prevalent lesion, accounted for 24 (32%) of the 76 defects. Next were atrial septal defects within the oval fossa (29%), patency of the arterial duct (17%), and ventricular septal defect (14%). There were only two cases of Fallot's tetralogy, one each of coarctation of the aorta and right-sided heart, respectively; and none of discordant ventriculo-arterial connections. Conclusion: The prevalence of cardiovascular malformations is high, at 57%, in our studied population of Arab children. Overall, 92% of the malformations are defects which are characterized by a left-to-right shunt, and the potential for pulmonary hypertension. These findings underscore the need for early detection and prompt, appropriate care. To achieve this, if feasible, all babies born with Down's syndrome should have echocardiography in the first month of life.

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