Scimittar Syndrome; Right time to operate? and mid-term results.

2021 ◽  
Author(s):  
Kenan Abdurrahman Kara ◽  
Ergi̇n Arslanoğlu ◽  
Fatih Tomrukçu ◽  
Abdullah Arif Yılmaz ◽  
Fatih Yiğit ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Pulmonary Arterial Pressure ◽  
Pulmonary Artery Hypertension ◽  
Scimitar Syndrome ◽  
Pulmonary Arterial ◽  
The Right ◽  
Vein Stenosis ◽  
Mid Term Results

Objectives: Scimitar syndrome is a combination of rare congenital cardiopulmonary anomalies that can occur in 3% to 6% of patients with a partial abnormal venous connection. The presence of accompanying cardiac anomalies in these patients and in cases such as severe hypoplasia of the right lung or accompanying pulmonary artery hypertension necessitate early surgery in early infancy. Patients and Methods: 9 patients with scimitar syndrome operated on in our pediatric cardiac surgery clinic from 2012 to 2020 were retrospectively examined in our study. The ages of the patients ranged from 1 to 47 years, with a mean of 18.11±14.44. 1 patient died and mortality was 11.11%. Of the patients, 4 were male (44.44%) and 5 were female (55.56%). Patients' pulmonary arterial pressure ranged from 0.15 to 94 mmHg, with a mean of 39.22 ±22.49. Results: Close to 25% scimitar vein stenosis or scimitar vein drainage occlusion has been reported in the postoperative period, mostly in the newborn group in the literature. 2 patients had non-critical stenosis during the 3rd year follow-up despite the absence of stenosis orocclusion during the first 2 years of follow-up of 9 patients we followed. Their surgical follow-up is still ongoing since they are asymptomatic. Conclusion: As a result, the course of the disease depends on the follow-up of the patient, the timing of the surgery, and the quality of the anastomosis. The follow-up and treatment of these patients will be more accurate in advanced centers experienced in scimitar surgery.

scholarly journals Epigenetic Regulation of Pulmonary Arterial Hypertension-Induced Vascular and Right Ventricular Remodeling: New Opportunities?

2020 ◽  
Vol 21 (23) ◽  
pp. 8901
Author(s):  
Jordy M. M. Kocken ◽  
Paula A. da Costa Martins
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Ventricular Remodeling ◽  
Pulmonary Artery Hypertension ◽  
Pulmonary Arterial ◽  
And Migration ◽  
Ventricle Hypertrophy ◽  
The Right ◽  
Disease Exposure

Pulmonary artery hypertension (PAH) is a rare chronic disease with high impact on patients’ quality of life and currently no available cure. PAH is characterized by constant remodeling of the pulmonary artery by increased proliferation and migration of pulmonary arterial smooth muscle cells (PASMCs), fibroblasts (FBs) and endothelial cells (ECs). This remodeling eventually leads to increased pressure in the right ventricle (RV) and subsequent right ventricle hypertrophy (RVH) which, when left untreated, progresses into right ventricle failure (RVF). PAH can not only originate from heritable mutations, but also develop as a consequence of congenital heart disease, exposure to drugs or toxins, HIV, connective tissue disease or be idiopathic. While much attention was drawn into investigating and developing therapies related to the most well understood signaling pathways in PAH, in the last decade, a shift towards understanding the epigenetic mechanisms driving the disease occurred. In this review, we reflect on the different epigenetic regulatory factors that are associated with the pathology of RV remodeling, and on their relevance towards a better understanding of the disease and subsequently, the development of new and more efficient therapeutic strategies.

scholarly journals Plasma exosomal miR-596: a novel biomarker predicts survival in patients with idiopathic pulmonary artery hypertension

2021 ◽  
Vol 49 (3) ◽  
pp. 030006052110023
Author(s):  
Yang Huang ◽  
Zuo-Gang Wang ◽  
Liang Tang ◽  
Su-Gang Gong ◽  
Yuan-Yuan Sun ◽  
...  
Keyword(s):  
Right Heart Catheterization ◽  
Pulmonary Artery Hypertension ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Distribution Analysis ◽  
Poor Overall Survival ◽  
Transmission Electron ◽  
Exosomal Mirnas ◽  
Pulmonary Arterial

Objective To determine if plasma exosomal microRNAs (miRNAs) can predict survival in patients with idiopathic pulmonary arterial hypertension (IPAH). Methods The study enrolled patients with IPAH that underwent right heart catheterization. Plasma was collected and exosomal miRNAs were extracted. Exosomes were evaluated using transmission electron microscopy, Western blot analysis and particle size distribution analysis. MiRNAs were evaluated using a miRNA microarray and validated using real-time polymerase chain reaction. Results This study included 12 patients with IPAH in the study group and 48 patients with IPAH in the validation group. The mean ± SD follow-up duration was 60.3 ± 35.4 months in the overall cohort. The levels of miR-596 were higher in the nonsurvivors compared with the survivors. The levels of miR-596 significantly correlated with survival time, mean right atrial pressure, pulmonary vascular resistance (PVR) and cardiac index. High levels of miR-596 and PVR were significantly associated with poor overall survival. Multivariate analysis demonstrated that exosomal miR-596 (hazard ratio [HR] = 2.119; 95% confidence interval [CI] 1.402, 3.203) and PVR (HR = 1.146; 95% CI 1.010, 1.300) were independent predictors of survival. Conclusions High levels of plasma exosomal miR-596 were significantly associated with disease severity and poor prognosis of patients with IPAH.

scholarly journals Multiple pericardial hematomas: a case report and mini-review in multimodality imaging

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Aninka Saboe ◽  
Ferdy Sanjaya ◽  
Raden Erwin Affandi Soeriadi ◽  
Euis Maryani ◽  
Nuraini Yasmin Kusumawardhani ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Cardiac Imaging ◽  
Single Photon ◽  
Multimodality Imaging ◽  
Photon Emission ◽  
Vena Cava ◽  
Intracardiac Thrombus ◽  
Surgical Closure ◽  
The Right

Abstract Background Pericardial hematoma is blood accumulation in the pericardial space. Although rare, it could arise in various conditions, such as after cardiac surgery. Clinical diagnosis of pericardial hematoma is implausible; thus, cardiac imaging plays a pivotal role in identifying this condition. We presented a case of multiple pericardial hematomas, which was found as an incidental finding in post-cardiac surgery evaluation. We highlighted the diagnostic challenge and the key features of multi-modality cardiac imaging in pericardial hematoma evaluation. Case presentation An asymptomatic, 35-years old male, who underwent surgical closure of secundum atrial septal defect (ASD) one month ago, came for routine transthoracic echocardiography evaluation. An intrapericardiac hematoma was visualized at the right ventricle (RV) 's free wall side. Another mass with an indistinct border was visualized near the right atrium (RA). This mass was suspected as pericardial hematoma differential diagnosed with intracardiac thrombus. Cardiac computed tomography (CT) scan showed both masses have an attenuation of 30–40 HU; however, the mass's border at the RA side was still not clearly delineated. Mild superior vena cava (SVC) compression and multiple mediastinal lymphadenopathies were also detected. These findings are not typical for pericardial hematomas nor intracardiac thrombus; hence another additional differential diagnosis of pericardial neoplasm was considered. We pursued further cardiac imaging modalities because the patient refused to undergo an open biopsy. Single-photon emission computer tomography (SPECT)/CT with Technetium-99 m (Tc-99 m) macro-aggregated albumin (MAA) and Sestamibi showed filling defect without increased radioactivity, thus exclude the intracardiac thrombus. Cardiac magnetic resonance imaging (MRI) reveals intrapericardial masses with low intensity of T1 signal and heterogeneously high intensity on T2 signal weighted imaged and no evidence of gadolinium enhancement, which concluded the diagnosis as subacute pericardial hematomas. During follow-up, the patient remains asymptomatic, and after six months, the pericardial hematomas were resolved. Conclusion Pericardial hematoma should be considered as a cause of pericardial masses after cardiac surgery. When imaging findings are atypical, further multi-modality cardiac imaging must be pursued to establish the diagnosis. Careful and meticulous follow-up should be considered for an asymptomatic patient with stable hemodynamic.

Pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 781-784
Author(s):  
Shahin Moledina ◽  
Bejal Pandya
Keyword(s):  
Quality Of Life ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Careful Assessment

Congenital heart disease is a major cause of pulmonary arterial hypertension (PAH) and this can largely be prevented by early repair. PAH in the presence of systemic-to-pulmonary communication, leads to shunt reversal and cyanosis, with multiple systemic consequences (Eisenmenger syndrome). Congenital heart disease patients with PAH are vulnerable and are at high risk from non-cardiac surgery, pregnancy, and inappropriate medical treatment (e.g. excessive venesection). Survival is reduced, but is better than in idiopathic PAH. Recommendations for surgery should be based on careful assessment by experts. Modern PAH pharmacotherapy is showing promise in improving quality of life.

scholarly journals A Case of Congenital Bronchial Pulmonary Arterial Malformation Successfully Treated by Embolization

2018 ◽  
Vol 02 (03) ◽  
pp. 201-204
Author(s):  
Ferdinand Chu ◽  
Ko Sit ◽  
King Kwok
Keyword(s):  
Bronchial Artery ◽  
Effective Means ◽  
Rare Condition ◽  
Embolic Material ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right ◽  
Arterial Malformation ◽  
Right Bronchial Artery

AbstractIdiopathic bronchial pulmonary arterial malformation (BPAM) is a very rare condition. The authors present a case of BPAM in which a right bronchial artery communicates with a main upper lobe branch of the right pulmonary artery. It was successfully treated by embolization in one setting. The patient remained asymptomatic and well during the follow-up period. The authors therefore conclude that if the embolic material/device is carefully chosen, it is a safe and effective means of treating BPAM.

scholarly journals Improved method for the catheterization of the right ventricle in a rat model of pulmonary artery hypertension

2020 ◽  
Vol 30 (4) ◽  
pp. 535-537
Author(s):  
Marcello Tontodonati ◽  
Debbie Ridley ◽  
René Remie ◽  
Peter Clements
Keyword(s):  
Right Ventricle ◽  
Jugular Vein ◽  
Disease Model ◽  
Pulmonary Artery Hypertension ◽  
Animal Disease ◽  
Improved Method ◽  
Novel Approach ◽  
Catheter Tip ◽  
The Right

Abstract Ventricle catheterization in the rat is widely practiced in cardiopulmonary research. The catheters deployed are either fluid filled or solid tip pressure or pressure–volume catheters. The access to the right ventricle is through the right jugular vein, most commonly without direct visualization such as fluoroscopy. Advancement of the catheter tip is aided by visualizing the pressure signals of the monitoring/recording systems used. This approach may present challenges due to various reasons, including the stiffness of new catheters, their dimensions or anatomical changes associated with the animal disease model. In this article, we present a novel approach, which has been optimized, successfully validated surgically and adopted in current projects. It has been shown to improve both the overall quality of the signals recorded and the time to access the right ventricle, thus reducing the overall time of surgery. The method presented in this article is safe, easy to reproduce and does not require additional skills compared to a more ‘standard’ approach.

Chronic lung lymph fistula that only drains the left lung

1995 ◽  
Vol 269 (4) ◽  
pp. R943-R947
Author(s):  
Y. Kikuchi ◽  
H. Nakazawa ◽  
D. L. Traber
Keyword(s):  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
Mediastinal Lymph Node ◽  
Pulmonary Veins ◽  
Pulmonary Arteries ◽  
Left Lung ◽  
Lymph Flow ◽  
Pulmonary Arterial ◽  
The Right ◽  
Arteries And Veins

We developed a chronic lung fistula that drains only the left lung, allowing for evaluation of injury in a single lung. To remove lymph drainage from the right lung into the caudal mediastinal lymph node, the right lower pulmonary ligament was severed. Pneumatic occluders were placed on the left pulmonary arteries and veins. To ensure that lymph drained from only the left lung, we increased the right pulmonary arterial pressure (RPAP) from 21.2 +/- 0.5 to 36.5 +/- 0.6 mmHg. The left pulmonary arterial pressure (LPAP) was kept at wedge pressure level for 1 h by inflating pneumatic occluders. Lymph flow from the left lung fistula was stable during this occlusion. Six hours after recovery was increased the LPAP from a baseline level of 19.1 +/- 1.0 to 36.4 +/- 0.9 mmHg and the RPAP from 21.2 +/- 0.5 to 38.0 +/- 0.8 mmHg for 2 h by inflating the pneumatic occluders on the left and right pulmonary veins. Lymph flow increased from 5.3 +/- 1.0 to 28.0 +/- 2.9 ml/h. Reflection coefficient was calculated at 0.80 +/- 0.02.

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