Alternative Hybrid Approach to Promote Native Pulmonary Artery Growth in Pulmonary Atresia VSD MAPCAs
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PROVIDER: S-EPMC9588454 | biostudies-literature | 2022 Oct
REPOSITORIES: biostudies-literature
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Project description:A 2-month-old baby with ventricular septal defect and pulmonary atresia was found to have coronary-to-pulmonary artery collaterals. Cardiac computed tomography confirmed the coronary collaterals and showed the absence of other systemic to pulmonary artery collaterals. Although these collaterals do not cause coronary ischemia, it is important to delineate them by accurate imaging to plan the appropriate surgical strategy.
| S-EPMC6146858 | biostudies-literature
Project description:BackgroundAsymptomatic, isolated cases of unilateral pulmonary artery atresia may present in adulthood with symptoms such as recurrent respiratory infections, dyspnea, hemoptysis, and pulmonary hypertension. Unlike previously reported patients that underwent surgical management for this pathology, the patient in this report had no chronic history of recurrent respiratory infections, dyspnea, or pulmonary hypertension, making a diagnosis prior to extensive imaging difficult.Case presentationA 55-year-old male presented to our emergency department (ED) with a 3-day history of recurrent cough with 2-3 tablespoons of hemoptysis per episode, chills, and occasional wheezing. A computed tomography angiography (CTA) was performed, which identified a congenital absence of the left pulmonary artery and a right-sided aortic arch. Hypertrophied left intercostal and bronchial arteries were noted to be perfusing the left lung. V/Q scan confirmed a heterogeneous distribution of gas throughout both lung fields with 97% perfusion to the right lung, but no visualization of the left lung on the perfusion images. Given extensive collateral blood supply to the left lung, interventional radiology performed a GELFOAM® embolization of the hypertrophied left bronchial artery and two parasitized arteries from the left subclavian artery to minimize intra-operative blood loss. This was immediately followed by a left thoracotomy, pneumonectomy, intercostal muscle flap placement, and bronchoscopy. The procedure was 360 min long with a total of 1500 cc blood loss that was salvaged and re-infused. No additional blood products were administered. The patient remained intubated post-operatively and was transferred to the surgical intensive care unit. His postoperative course was complicated by troponin leak, rhabdomyolysis, delirium, and ileus, all of which resolved over time. He was discharged home on postoperative day seven and continues to do well one-year later.ConclusionsThe patient in this report presented with several episodes of isolated hemoptysis but unlike previously reported cases of unilateral pulmonary artery atresia, he had no history of recurrent respiratory infections, dyspnea, or pulmonary hypertension. Although unilateral pulmonary artery atresia is a rare diagnosis, in patients with unexplained, isolated hemoptysis, further examination of the vasculature may be warranted, and surgical management may be beneficial in appropriate, symptomatic patients.
| S-EPMC10311879 | biostudies-literature
Project description:Adult presentation of unilateral pulmonary artery atresia in association with contralateral branch pulmonary stenosis is rare. We present the case of a quadragenarian, who manifested with right ventricular failure and hemoptysis. This report discusses the diagnostic workup and therapeutic options along with a brief overview of the concerned literature.
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Project description:IntroUnilateral pulmonary artery atresia (UPAA), while encountered frequently in the congenital cardiac anomaly cohort, is occasionally diagnosed in adulthood after typical symptoms of hemoptysis, pulmonary infection, or as an incidental finding on contrast CT scan. Due to its rarity, a brief discussion of UPAA and its treatment is warranted.Case reportA 35 year old male presented with three days of hemoptysis. After diagnosis of right UPAA, he underwent angioembolization of 6 large systemic collaterals supplying his right lung, followed by right pneumonectomy. He was discharged on post-operative day 3, and at follow up 6 weeks later was doing well with minimal residual incisional pain and excellent pulmonary reserve.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.
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Project description:We describe the use of direct percutaneous cardiac access to recanalize an atretic right pulmonary artery in an adolescent with complex congenital heart disease and right heart failure. This case highlights the problems associated with loss of central venous access and potential advantages of a direct cardiac approach to catheterization. (Level of Difficulty: Intermediate.).
| S-EPMC8310936 | biostudies-literature
Project description:BackgroundHypoplastic right heart syndrome with pulmonary atresia is a rare cyanotic heart disease with poor prognosis requiring urgent intervention to establish the pulmonary blood flow. Pulmonary blood flow is achieved by BT shunt or percutaneous techniques like PDA stenting or pulmonary valve perforation. Various series have shown that early surgical intervention causes high mortality in these patients. Pulmonary valve perforation is a suitable, physiological alternative to surgical techniques in selected patients.Case presentationWe report a case of hypoplastic right heart syndrome with pulmonary atresia and restrictive VSD presenting with cyanosis from birth and underwent pulmonary valve perforation successfully.ConclusionDuct-dependent pulmonary circulation is a pediatric emergency, palliative procedure for establishing adequate pulmonary blood flow is essential early in the life. In the management of duct-dependent pulmonary circulation, RVOT perforation is an effective and safe option in suitable high-risk subgroups. The induced pulmonary regurgitation along with established physiological antegrade flow would be beneficial in the remodeling of tripartite/hypertrophied small RV.
| S-EPMC7524925 | biostudies-literature
Project description:Pulmonary atresia with ventricular septal defect (PA/VSD) is a rare congenital heart disease (CHD) characterized by a lack of luminal continuity and blood flow from either the right ventricle or the pulmonary artery, together with VSDs. The prevalence of PA/VSD is about 0.2% of live births and approximately 2% of CHDs. PA/VSD is similar to tetralogy of Fallot (TOF) in terms of structural and pathological characteristics. The pathogenesis of these two CHDs remains incompletely understood. It was previously reported that N-myc downstream-regulated gene (NDRG)4 is required for myocyte proliferation during early cardiac development. In the present study, we enrolled 80 unrelated patients with PA/VSD or TOF and identified a probably damaging variant p.T256M of NDRG4. The p.T256M variant impaired the proliferation ability of human cardiac myocytes (hCM). Furthermore, the p.T256M variant resulted in G1 and G2 arrest of hCM, followed by an increase in p27 and caspase-9 expression. Our results provide evidence that the p.T256M variant in NDRG4 is a pathogenic variant associated with impaired hCM proliferation and cell-cycle arrest and likely contributes towards the pathogenesis of PA/VSD and TOF.
| S-EPMC7876499 | biostudies-literature
Project description: Not available
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Project description:Pulmonary artery catheters (PACs) can provide extremely valuable objective data in select patients. They are usually advanced by floatation of balloon tip along the normal blood flow and their placement is confirmed under pressure waveform guidance. Imaging such as fluoroscopy is often employed in low flow states and in cardiac catheterization suite to reduce the failure rate and time to wedge; but is not readily available at bedside. In critically ill patient, bedside insertion is feasible but can be complicated by repeated attempts to float the balloon tip through various cardiac chambers. Point of care ultrasound can be used to visualize the balloon tip of PAC inside the cardiac chambers alongside the confirmatory pressure waveform changes.
| S-EPMC9151730 | biostudies-literature
Project description:Vascular atresia are often treated via transcatheter recanalization or surgical vascular anastomosis due to congenital malformations or coronary occlusions. The cellular response to vascular anastomosis or recanalization is, however, largely unknown and current techniques rely on restoration rather than optimization of flow into the atretic arteries. An improved understanding of cellular response post anastomosis may result in reduced restenosis. Here, an in vitro platform is used to model anastomosis in pulmonary arteries (PAs) and for procedural planning to reduce vascular restenosis. Bifurcated PAs are bioprinted within 3D hydrogel constructs to simulate a reestablished intervascular connection. The PA models are seeded with human endothelial cells and perfused at physiological flow rate to form endothelium. Particle image velocimetry and computational fluid dynamics modeling show close agreement in quantifying flow velocity and wall shear stress within the bioprinted arteries. These data are used to identify regions with greatest levels of shear stress alterations, prone to stenosis. Vascular geometry and flow hemodynamics significantly affect endothelial cell viability, proliferation, alignment, microcapillary formation, and metabolic bioprofiles. These integrated in vitro-in silico methods establish a unique platform to study complex cardiovascular diseases and can lead to direct clinical improvements in surgical planning for diseases of disturbed flow.
| S-EPMC8823098 | biostudies-literature