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Survival outcomes in EIF2AK4 mutation-associated pulmonary arterial hypertension: seeking clarity in contrast.
Eur Heart J Case Rep
November 2024
National Pulmonary Hypertension Centre, Royal Brompton Hospital, Part of GSTT Foundation Trust, Sydney Street, London SW3 6NP, UK.
Article Synopsis
- Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary arterial hypertension (PAH) linked to mutations in the EIF2AK4 gene, particularly presenting severe outcomes for those with homozygous mutations.
- This case study highlights two patients with homozygous EIF2AK4 mutations who responded exceptionally well to pulmonary vasodilator therapy and showed stable health over a 2 to 10-year follow-up period.
- The findings suggest that intrapulmonary shunts (IPS) might help relieve pressure on the right ventricle while maintaining oxygen levels, indicating a potentially better prognosis for PVOD patients compared to classical PAH cases.
Pulmonary CT perfusion robustly measures cardiac output in the context of multilevel pulmonary occlusion: a porcine study.
Eur Radiol Exp
March 2024
Department of Diagnostic and Interventional Radiology, Uniklinik RWTH Aachen, Aachen, Germany.
Background: To validate pulmonary computed tomography (CT) perfusion in a porcine model by invasive monitoring of cardiac output (CO) using thermodilution method.
Methods: Animals were studied at a single center, using a Swan-Ganz catheter for invasive CO monitoring as a reference. Fifteen pigs were included.
Influence of Fractional Inspired Oxygen Tension on Lung Perfusion Distribution, Regional Ventilation, and Lung Volume during Mechanical Ventilation of Supine Healthy Swine.
Anesthesiology
April 2024
Laboratorio de Pneumologia LIM-09, Disciplina de Pneumologia, Instituto de Cardiologia (Incor), Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
Background: Lower fractional inspired oxygen tension (Fio2) during general anesthesia can reduce lung atelectasis. The objectives are to evaluate the effect of two Fio2 (0.4 and 1) during low positive end-expiratory pressure (PEEP) ventilation over lung perfusion distribution, volume, and regional ventilation.
View Article and Find Full Text PDFChanges in lung mechanics and ventilation-perfusion match: comparison of pulmonary air- and thromboembolism in rats.
BMC Pulm Med
January 2024
Department of Medical Physics and Informatics, University of Szeged, 9 Korányi fasor, Szeged, H-6720, Hungary.
Background: Pulmonary air embolism (AE) and thromboembolism lead to severe ventilation-perfusion defects. The spatial distribution of pulmonary perfusion dysfunctions differs substantially in the two pulmonary embolism pathologies, and the effects on respiratory mechanics, gas exchange, and ventilation-perfusion match have not been compared within a study. Therefore, we compared changes in indices reflecting airway and respiratory tissue mechanics, gas exchange, and capnography when pulmonary embolism was induced by venous injection of air as a model of gas embolism or by clamping the main pulmonary artery to mimic severe thromboembolism.
View Article and Find Full Text PDFPartial anomalous venous connection with intrapulmonary dual drainage: Transcatheter treatment of a rare entity.
Ann Pediatr Cardiol
September 2023
Department of Surgery, Division of Pediatric Cardiovascular Surgery, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota.
Partial anomalous pulmonary venous return (PAPVR) is a rare congenital condition, and dual-drainage connection PAPVR to the left atrium has been reported in a few cases in the literature; in which cases, percutaneous catheterization was successfully used in lieu of surgery. We, hereby, describe a 7-month-old boy with a functional single-ventricle physiology with dual drainage of the left upper pulmonary vein to the left atrium and the innominate vein. Appropriate recognition of this entity allowed safe occlusion of the anomalous draining vein.
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