Pulmonary Veins: Not Always Where You Expect Them.
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ABSTRACT:
SUBMITTER: Sunthankar KI
PROVIDER: S-EPMC10954582 | biostudies-literature | 2024 Mar
REPOSITORIES: biostudies-literature
Publications
Pulmonary Veins: Not Always Where You Expect Them.
Sunthankar Kathryn I KI Weingarten Angela J AJ Doyle Thomas P TP Nicholson George T GT Sunthankar Sudeep D SD
CASE (Philadelphia, Pa.) 20240308 3Part A
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Project description:Partial anomalous pulmonary venous connection (PAPVC) is an extremely rare congenital condition where one or more of the pulmonary veins are connected to the venous circulation. Although initially suspected with unexplained right ventricular enlargement on transthoracic echocardiography (TTE), cardiac MRI is able to delineate the anatomical variant. We present a case of a 65-year-old male diagnosed with left sided PAPVC using multimodality cardiac imaging.
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Project description:BackgroundPulmonary Vein Thrombosis (PVT) is a rare and underdiagnosed entity produced by local mechanical nature mechanisms, vascular torsion or direct injury to the vein. PVT has been described in clinical cases or small multicenter series mainly in relation to pulmonary vein stenosis, metastatic carcinoma, fibrosing mediastinitis, as an early surgical complication of lung transplantation lobectomy and radiofrequency ablation performed in patients with atrial fibrillation, although in some cases the cause is not known.CaseWe report the case of a 57 years old male with history of atrial fibrillation treated by radiofrequency ablation who was admitted in our center because of a two-week history of consistent pleuritic pain in the left hemithorax and low-grade hemoptysis and a lung consolidation treated as a pneumonia with antibiotic but not responding to medical therapy. In view of the poor evolution of the patient, computed tomography angiography was performed with findings of PVT and secondary venous infarction and anticoagulation therapy was optimized. At the end, pulmonary resection was performed due to hemorrhagic recurrence.ConclusionPVT remains a rare complication of radiofrequency ablation and other procedures involving pulmonary veins. Clinical suspicion and early diagnosis is crucial because is a potentially life-threatening entity.
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Project description:Although Doppler analysis of pulmonary veins (PVs) is crucial in the assessment of cardiac hemodynamics, there is controversy regarding individual anatomical PV imaging with transthoracic echocardiography (TTE). This report is a discussion of how to image PVs accurately using TTE. To resolve any contradiction, multiple TTE images were obtained during the selective catheterization of the PV in patients undergoing atrial fibrillation ablation procedure. Fluoroscopic images were used as a reference for the identification of each PV and simultaneous echocardiographic imaging of the catheter positioned in the distal PV was used for accurate anatomical localization of the ostium and distal part of the PV.
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Project description:Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a lethal lung developmental disorder caused by heterozygous point mutations or genomic deletion copy-number variants (CNVs) of FOXF1 or its upstream enhancer involving fetal lung-expressed long noncoding RNA genes LINC01081 and LINC01082. Using custom-designed array comparative genomic hybridization, Sanger sequencing, whole exome sequencing (WES), and bioinformatic analyses, we studied 22 new unrelated families (20 postnatal and two prenatal) with clinically diagnosed ACDMPV. We describe novel deletion CNVs at the FOXF1 locus in 13 unrelated ACDMPV patients. Together with the previously reported cases, all 31 genomic deletions in 16q24.1, pathogenic for ACDMPV, for which parental origin was determined, arose de novo with 30 of them occurring on the maternally inherited chromosome 16, strongly implicating genomic imprinting of the FOXF1 locus in human lungs. Surprisingly, we have also identified four ACDMPV families with the pathogenic variants in the FOXF1 locus that arose on paternal chromosome 16. Interestingly, a combination of the severe cardiac defects, including hypoplastic left heart, and single umbilical artery were observed only in children with deletion CNVs involving FOXF1 and its upstream enhancer. Our data demonstrate that genomic imprinting at 16q24.1 plays an important role in variable ACDMPV manifestation likely through long-range regulation of FOXF1 expression, and may be also responsible for key phenotypic features of maternal uniparental disomy 16. Moreover, in one family, WES revealed a de novo missense variant in ESRP1, potentially implicating FGF signaling in the etiology of ACDMPV.
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Project description:Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a rare, lethal cause of neonatal respiratory failure and persistent pulmonary hypertension. We present a presumptive prenatal diagnosis of ACDMPV based on chorionic villus sampling of a FOXF1 mutation in a fetus with extra-pulmonary anomalies often associated with ACDMPV.
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Project description:BackgroundPositional indications for sublobar resection have been increasingly focused by clinical surgeons while the surgical strategies for cases involving inter-multisegmental veins (IMSVs) remain underreported. We want to further clarify the positional indications for sublobar resection in patients with clinical T1a-bN0 non-small cell lung cancer (NSCLC).MethodsThe clinical data of 686 patients from August 2021 to July 2022 were retrospectively analyzed. In the three-dimensional (3D) reconstruction images, we analyzed the prevalence and drainage patterns of typical IMSVs, specifically focusing on the lateral vein (Vl) in the upper lobes and the branches of the superior segmental vein (V6b) in the lower lobes. The potential association between lesion locations and surgical strategy was also analyzed.ResultsThe prevalence of Vl, V6b2, and V6b3 was 58.5% (231/395), 98.3% (286/291), and 25.1% (73/291), respectively. Vl mainly drained into V2a+b (70/110, 63.6%) on the right and into V1+2b+c (72/121, 59.5%) on the left. V6b2 and V6b3 mainly converged with other branches of the superior segmental vein. Limited resection was more feasible when the 2 cm simulated cutting margin of nodules did not involve IMSVs, or when lesions were located in the outer region. Multivariable logistic regression analyses identified four independent predictors for surgical procedure selection: (I) whether the 2 cm simulated cutting margin involves IMSVs; (II) diameter; (III) consolidation-to-tumour ratio (CTR); and (IV) depth ratio.ConclusionsIMSVs exhibited high prevalences, with Vl showing diverse drainage patterns, while V6b2 and V6b3 displayed little variation. Depth ratio and the lesion's relative location to the IMSVs were identified as longitudinal and transverse positional indications, respectively, for sublobar resection in patients with clinical T1a-bN0 NSCLC.
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