Surgical repair of peripheral pulmonary artery stenosis in patients without williams or alagille syndromes

Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was a retrospective review of 30 patients who underwent surgical repair for peripheral pulmonary artery stenosis. Concomitant anatomical diagnoses in 20 patients (67%) included: supravalvar aortic stenosis (n=8), tetralogy of Fallot (n=4), d-transposition of the great arteries (n=2), truncus arteriosus (n=2), hypoplastic left heart syndrome (n=2), ventricular septal defect (n=1), and patent ductus arteriosus (n=1).
Source: Seminars in Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: CONGENITAL – Original Submission Source Type: research

Related Links:

Discussion Congenital heart diseases (CHD) are malformations of the heart and great vessels. It occurs in about 5-8/1000 live births. Cyanotic congenital heart disease is often noted perinatally because of cyanosis, respiratory distress and/or poor feeding or other distress type problems. A review can be found here. Acyanotic congenital heart disease (ACHD) can present at birth but often is seen in older children or adults unless the lesions are severe, especially obstructive lesions. Severe lesions may also cause cyanosis and distress type problems in patients also. Shunting lesions cause problems by diverting blood flo...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Conclusion: In a unit were complex pediatric congenital heart surgery started twenty years ago, an increasing adult CHD workload was documented. Referral came predominantly from cities around the unit, most patients had low complexity defects and were in functional class I, a significant loss of follow-up was documented, and the death of patients was mainly due to the heart defect.
Source: Revista Brasileira de Cirurgia Cardiovascular - Category: Cardiovascular & Thoracic Surgery Source Type: research
ri S, Manohar K, Marwah A, Mishra S, Mohanty SR, Murthy KS, Suresh PV, Radhakrishnan S, Rajashekar P, Ramakrishnan S, Rao N, Rao SG, Reddy CH, Sharma R, Shivaprakasha K, Subramanyan R, Kumar RS, Talwar S, Tomar M, Verma S, Raju V, Working group on Management of Congenital Heart Disease in India Abstract JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital he...
Source: Indian Pediatrics - Category: Pediatrics Authors: Tags: Indian Pediatr Source Type: research
This Medicine MCQ Test Series contains 20 questions which can be attempted over 40 seconds each. After submission, answers and discussion will be displayed. Medicine MCQ Test Series 1 Time limit: 0 Quiz-summary 0 of 20 questions completed Questions: 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 Information ...
Source: Cardiophile MD - Category: Cardiology Authors: Tags: Medicine MCQ - CVS Source Type: blogs
ConclusionsECMO can be used as a bridge to heart valve or septal defect surgery in severely decompensated patients. Through recovery of end-organ function, ECMO may allow surgical correction of structural heart disease in patients considered inoperable or convert a salvage situation to an elective operation.
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
CONCLUSIONS: ECMO can be used as a bridge to heart valve or septal defect surgery in severely decompensated patients. Through recovery of end-organ function, ECMO may allow surgical correction of structural heart disease in patients considered inoperable or convert a salvage situation to an elective operation. PMID: 30236527 [PubMed - as supplied by publisher]
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Ann Thorac Surg Source Type: research
It is needless to say that heart disease in pregnancy is a challenge for the obstetrician and the cardiologist. Hemodynamic changes in pregnancy and labour can adversely affect many of the significant cardiac lesions. Increase in blood volume and heart rate are the important factors during pregnancy. In general stenotic lesions and pulmonary hypertension are poorly tolerated, while regurgitant lesions are better tolerated. Specific risks like aortic dissection and rupture are there for coarctation of aorta. Several risk stratification schemes have been developed for assessing the risk of pregnancy with heart disease over ...
Source: Cardiophile MD - Category: Cardiology Authors: Tags: General Cardiology CARPREG II risk scoring CARPREG risk stratification mWHO classification ZAHARA risk score Source Type: blogs
Conclusions: The majority of isolated bicuspid aortic valve cases without aortic stenosis did not require surgical intervention. Outcomes for cases requiring repair/replacement were relatively benign.
Source: Annals of Pediatric Cardiology - Category: Cardiology Authors: Source Type: research
Abstract BackgroundBicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. MethodsThe Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention. ResultsOverall, 1010 patients with BAV...
Source: Congenital Heart Disease - Category: Cardiology Authors: Tags: Original Article Source Type: research
Time limit: 0 Quiz-summary 0 of 30 questions completed Questions: 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 Information This new test series requires ...
Source: Cardiophile MD - Category: Cardiology Authors: Tags: Cardiology Cardiology MCQ DM / DNB Cardiology Entrance Featured Source Type: blogs
More News: Aortic Stenosis | Cardiology | Cardiovascular | Cardiovascular & Thoracic Surgery | Gastroschisis Repair | Genetics | Heart | Heart Disease | Hole in the Heart | Study | Ventricular Septal Defect