Living Donor Liver Transplantation in Biliary Atresia Children with Pulmonary Hypertension.

CONCLUSION: LDLT is a safe procedure in a selected group of BA patients with PH, however, further long-term clinical investigations and mechanical researches are needed. PMID: 31588186 [PubMed - in process]
Source: International Journal of Medical Sciences - Category: Biomedical Science Tags: Int J Med Sci Source Type: research

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We describe 3 children with complex congenital heart defects who have a variable degree of segmental pulmonary hypertension and discuss management strategies and the role of interventional and/or pulmonary hypertension targeted therapies. PMID: 32987636 [PubMed - in process]
Source: Medicina (Kaunas) - Category: Universities & Medical Training Authors: Tags: Medicina (Kaunas) Source Type: research
This study aimed to identify preoperative hemodynamic factors associated with segmental pulmonary hypertension after intracardiac repair in patients with PA/VSD/MAPCAs. This study included 12 patients with PA/VSD/MAPCAs who underwent unifocalization and intracardiac repair from 2009 at our institution. Hemodynamic measurements of preoperative and postoperative cardiac catheterization were collected. Patients were considered to have pulmonary hypertension if the mean pulmonary pressure measured postoperatively at any peripheral pulmonary artery was  ≥ 20 mmHg. The data were compared between patients wi...
Source: Pediatric Cardiology - Category: Cardiology Source Type: research
We report the first presentation of bronchial varices and haemoptysis in a child, six years after a Fontan procedure for tricuspid atresia. She had tortuous mediastinal and transpleural arterial collaterals on imaging and subsequently underwent embolization of these collaterals with haemoptysis settling after this. AbstractTracheal and bronchial varices are rarely found in children. However, they have been described in adults with failing Fontan circuits or secondary to vascular pathology, such as portal and pulmonary hypertension. We report the presentation of haemoptysis and bronchial varices in a child, six years after ...
Source: Respirology Case Reports - Category: Respiratory Medicine Authors: Tags: Case Report Source Type: research
We report a 3-year-old boy with recurrent hemoptysis for 2 years. The child had tachycardia, tachypnea, tender hepatomegaly, and left-sided decreased air entry with crepitations. Coagulation and Koch's workup was negative. X-ray of the chest showed a small left hemithorax. Computed tomography with angiography showed left unilateral PVA with pulmonary artery hypoplasia and dysplastic left lung. The child has been posted for left pneumonectomy. Although uncommon, PVA should be diagnosed early to prevent life-threatening complications such as hemoptysis and pulmonary hypertension. The patient can be managed conservatively...
Source: Lung India - Category: Respiratory Medicine Authors: Source Type: research
Publication date: Available online 11 September 2019Source: JACC: Heart FailureAuthor(s): Karen Sliwa, Lucia Baris, Christoph Sinning, Elvin Zengin-Sahm, Lina Gumbiene, Israa F. Yaseen, Ghada Youssef, Mark Johnson, Hasan Al-Farhan, Malgorzata Lelonek, Roger Hall, Jolien Roos-HesselinkAbstractObjectivesThe purpose of this work was to study maternal and fetal outcomes of women with uncorrected congenital heart disease (CHD).BackgroundGlobally, CHD is an important cause of maternal morbidity and mortality in women reaching reproductive stage. Data are lacking from larger cohorts of women with uncorrected CHD.MethodsThe 10-yea...
Source: JACC: Heart Failure - Category: Cardiology Source Type: research
Publication date: Available online 11 September 2019Source: JACC: Heart FailureAuthor(s): Karen Sliwa, Lucia Baris, Christoph Sinning, Elvin Zengin-Sahm, Lina Gumbiene, Israa F. Yaseen, Ghada Youssef, Mark Johnson, Hasan Al-Farhan, Malgorzata Lelonek, Roger Hall, Jolien Roos-HesselinkAbstractBackgroundGlobally, congenital heart disease (CHD) is an important cause of maternal morbidity and mortality in women reaching reproductive stage. There is lack of data from larger cohorts of women with uncorrected CHD.ObjectivesTo study maternal and fetal outcome of women with uncorrected CHD.MethodsAn analysis of 10-year data from th...
Source: JACC: Heart Failure - Category: Cardiology Source Type: research
The objective of this article is to present a case of congenital bronchial atresia manifesting as pulmonary hypertension in a 66 year-old patient. While doing so, we highlight the relevant knowledge accrued in medical literature with regards this rare condition. Finally, with the revelation that congenital bronchial atresia may cause severe pulmonary hypertension later in life, perhaps a less conservative approach to management may be warranted in younger adults and children with this condition.
Source: Respiratory Medicine Case Reports - Category: Respiratory Medicine Source Type: research
ConclusionWith low operative mortality for often very severe heart disease, and good long-term follow-up, the management of children from countries without cardiac surgery is justified by survival similar to that of Western cohorts - except for acquired valvulopathies, presumably because of the difficulty of anticoagulant treatment. Thus our humanitarian action must continue, and improve for an optimal management of the disinherited populations.
Source: Archives of Cardiovascular Diseases Supplements - Category: Cardiology Source Type: research
ConclusionsUPAA presents classically with hemoptysis, but also with pneumonia, pulmonary hypertension, or incidentally. Management includes selective collateral embolization, pneumonectomy, or medical management directed towards decreasing pulmonary hypertension in patients unable to tolerate pneumonectomy due to comorbidities. Pneumonectomy in these patients is characterized by dense and hypervascular adhesions, with large volume blood loss expected during adhesiolysis, which can be decreased with pre-operative embolization. Outcomes are typically excellent in otherwise healthy patients.
Source: Respiratory Medicine Case Reports - Category: Respiratory Medicine Source Type: research
Conclusion: PPHH may be an underdiagnosed complication in children undergoing surgery for oesophageal atresia. These children must be monitored closely for symptoms of hypoglycaemia and if there are concerns must be screened for possible PPHH. Our cases demonstrate that continuous feeding regimens might be the only therapeutic option, until PPHH gradually lessens in intensity over time.Horm Res Paediatr
Source: Hormone Research in Paediatrics - Category: Endocrinology Source Type: research
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