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Pulmonary arterial hypertension, a common complication in patients with congenital heart defects with left-to-right shunt who present late is one of the principal determinants of the clinical manifestations, the course and the feasibility of surgical repair. The objective is to study the pulmonary arterial pressures and the pulmonary histopathologi...
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... septal lymphocytosis ( Fig. 1) was the most common parenchymal change and was found in 50% of cases. Alveolar septal fibrosis (Fig. 2) was found in 25% of cases. Alveolar hemorrhage (Fig. 3) and Alveolar hemosiderosis (Fig. 4) were found in 40% and 30% cases respectively. Pleural lymphocytosis and Pleural fibrosis were found in 40% and 15% cases respectively. There was no statistically significant correlation between pulmonary parenchymal changes and the intra-operative mean PAP (pre-and post-repair), the age of the patient at the time of surgical repair, the clinical profile of the patient, the radiological findings, type of congenital cardiac defect, and the size of the ...
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Citations
... signifying a direct correlation between mPAP and PVR. Our findings were comparable to that of Reddy et al 24 . M Vogel et al 25 . ...
... signifying a direct correlation between mPAP and PVR. Our findings were comparable to that of Reddy et al 24 . M Vogel et al 25 . ...
... signifying a direct correlation between mPAP and PVR. Our findings were comparable to that of Reddy et al 24 . M Vogel et al 25 . ...
... The age ranged from 4 months to 25 years (mean age-5.8 years) 9 . on the other hand Yau KI, Fang LJ and Wu MH enrolled twenty-six infants (13 males, l3 females) with CHD and left-to-right shunt from lower age group [9 days to 11 months (median, 3.3 months)] 10 . ...
... Differential cyanosis/clubbing, evidence of longstanding pulmonary hypertension with reversal of shunt was not seen in any patient. A loud second heart sound due to accentuation of pulmonary component, a presumptive evidence of pulmonary arterial hypertension was found in 3 patients (15%) 9 . In Bangladesh major clinical presentations of patients with CHD were breathlessness (60%), fatigue (54.8%), cough (43.5%), poor weight gain (41.7%), recurrent chest infection (34.8%), feeding problem (26.1%), cyanosis (20%), clubbing All the children above 5 years of age [9 (18.0%)] had moderate to severe PAH. ...
... The other had Ostium secundum type with 34 mm size. One patient had mixed lesion with ventricular septal defect of 6 mm size and patent ductus arteriosus of 7 mm size9 . In Rajshahi Medical College Hospital, commonest congenital heart disease was VSD (42.6%) followed by TOF (18.3%), ...
The Normal Pulmonary Artery (PA) systolic pressure of children and adults is < 30 mm Hg and the mean PA pressure is < 25 mm Hg at sea level. Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure > 25 mm Hg at rest or > 30 mm Hg during exercise. An increase in pulmonary flow, vascular resistance, or both can result in pulmonary hypertension. Pulmonary Arterial Hypertension (PAH) commonly arises in patients with Congenital Heart Diseases (CHD) are usually associated with increased pulmonary blood flow. Greater number of patients with Pulmonary Arterial Hypertension (PAH), associated with congenital heart disease, are now surviving into adulthood, many with increasingly complex cardiac defects. Patients with cardiac defects which result in left-to-right shunting tend to develop PAH, owing to the increased shear stress and circumferential stretch induced by pulmonary blood flow, which leads to endothelial dysfunction and progressive vascular remodeling followed by vascular resistance. Pulmonary hypertension in association with congenital heart diseases is seen in large systemicto- pulmonary communications such as Ventricular Septal Defect (VSD), Patent Ductus Arteriosus (PDA) atrioventricular septal defects, aorticopulmonary window defect etc. Pulmonary hypertension associated with large L-R shunt lesions (e.g. VSD, PDA) is called Hyperkinetic Pulmonary Hypertension (PH). It is the result of an increase in pulmonary blood flow, a direct transmission of the systemic pressure to the pulmonary artery, and compensatory pulmonary vasoconstriction. Hyperkinetic PH is usually reversible if the cause is eliminated before permanent changes occur in the pulmonary arterioles. If large L-R shunt lesions are left untreated, irreversible changes take place in the pulmonary vascular bed, with severe pulmonary hypertension and cyanosis due to a reversal of the L-R shunt. This stage is called Eisenmenger syndrome or Pulmonary Vascular Obstructive Disease (PVOD). Surgical correction is not possible at this stage. Due to lack of formal study which of the L-R congenital shunt is more commonly associated with the development of pulmonary hypertension is not known the cross-sectional study therefore intends to find the pattern of congenital L-R heart diseases commonly attributed to the development pulmonary hypertension. The diagnosis of the abovementioned congenital heart disease will be made by echocardiography. In this study, most of the participant [22 (44.0%)] were in the ‘< 1 years’ age group, female were proportionately higher, most patients presented with cough and difficulty in breathing, dyspnoea and tachycardia were the most common clinical findings, murmur mostly systolic were found, Eisenmenger syndrome was not found in any child, ASD was the most common congenital anomaly. About four-fifth of the participants had single congenital anomaly. Two-fifth of the participant was found having severe PAH. Significant correlation were revealed between age group and Pulmonary arterial pressure (R= 0.775), Status of PAH was found significantly different (p< 0.05) in age groups and presence of number of anomalies.Chatt Maa Shi Hosp Med Coll J; Vol.14 (2); Jul 2015; Page 31-37
... signifying a direct correlation between mPAP and PVR. Our findings were comparable to that of Reddy et al 24 . M Vogel et al 25 . ...
Background: Development of pulmonary vascular disease (PVD) has been considered a risk factor in the long-term course of ASD (atrial septal defect). Lung biopsy remains the gold standard to confirm the degree of severity in pulmonary vascular disease in patients with isolated ASD. Objective: To determine the effect on natural course on pulmonary vascular diseases in isolated ASD patients at high altitude .To assess the histological degree of pulmonary vascular diseases in isolated ASD patients. Material and Methods: This study was carried in the Department of Cardiovascular and Thoracic Surgery Sher-i-Kashmir Institute of Medical Sciences between 1 st September 2010 to 31 st November 2012.Patients with isolated ASD was included in the study. Isolated ASD Patients. Ostium secundum (OS) type. Patients residing at more than 3,500ft above sea-level. All patients were subjected to cardiac cath prior to surgery. Pulmonary vascular resistance was calculated in all patients. Lung Biopsy (which was taken during repair of ASD before patient was put on cardio pulmonary bypass) was sent for histopathological examination. Histopathological examination of the specimen was done by a single histopathologist. Severity of the pulmonary vascular disease was assigned a Score between 1 -4. Results: histopathological score of one was seen in none whereas 5.6% of the patients (n=2) had a histopathological score of 2; 44.4 % (n=8) had a histological score of 3 and 50% (n=9) had a histological score of 4. Most of our patients (94.4%; n=17) had a higher histopathological score of 3 & 4. Conclusion: No formal study has been conducted so far to analyze the effect of high altitude on pulmonary vascular disease in Atrial Septal defect. All of our patients had established pulmonary vascular changes at the time of presentation. Severity of the pulmonary vascular disease as proved by histopathological
