Pulmonary Artery Dilatation Due to Pressure or Volume Overload in Congenital Heart Disease.

Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery (MPA) diameter and MPA/ascending aorta (Ao asc) ratio, by echocardiography (ECHO) and computer tomography or magnetic resonance imaging (CT/MRI). Results: In MPA diameter, significant differences between PAH-CHD, rTOF/PR, and NORMAL were found (median): 37 vs. 27 vs. 21 mm (p < 0.0001). In MPA/Ao asc ratio, there was a difference between PAH-CHD and NORMAL (median): 1.3 vs. 0.8 (p < 0.0001), but not between rTOF/PR and NORMAL: 0.74 vs. 0.8 (p = 0.3). Significant MPA dilatation (>40 mm) was present: in PAH-CHD, 35% (ECHO) and 76.9% (CT/MRI) of patients, while in rTOF/PR, 3.1% (ECHO) and 7.8% (CT/MRI). Severe MPA dilatation (>50 mm) occurred only in PAH-CHD: 16.7% (ECHO) and 31.4% (CT/MRI), while not in rTOF/PR. There was a significant correlation between ECHO and CT/MRI measurements, but ECHO was underestimated in all parameters. Conclusions: MPA dilatation due to pressure overload is more frequent and more severe; volume overload also leads to MPA dilatation but is less severe. The MPA/Ao asc ratio is not reliable for MPA dilatation estimation in rTOF/PR.

Risk of chronic thromboembolic pulmonary hypertension after splenectomy.

OBJECTIVES: The purpose of the clinical study was to evaluate the risk of chronic thromboembolic pulmonary hypertension (CTEPH) after splenectomy and to analyze some biochemical and coagulation parameters. BACKGROUND: CTEPH caused by incomplete resolution of thromboemboli and irreversible remodeling of the pulmonary arteries is a progressive, and without treatment a fatal disease. Although the definite etiopathophysiology is not quite perfectly researched, numerous clinical conditions associated with CTEPH as history of pulmonary embolism, infected ventriculoatrial shunts or permanent intravascular devices, high-dose thyroid hormone replacement, malignancy and chronic inflammatory diseases, including osteomyelitis, inflammatory bowel diseases, are well accepted. These factors also include splenectomy. METHODS: We performed a prospective follow-up of patients after splenectomy in the period of 5 years (2017-2022). The study population consisted of 62 adult post-splenectomy patients, who were divided into 3 groups based on the cause of the splenectomy - trauma, haematologic diseases, and others. The study population was analyzed in terms of gender, age, cause of splenectomy, blood group, clinical risk factors and thrombophilic conditions. Some basic haemocoagulation parameters and selected coagulation and biochemical parameters were analyzed. All patients underwent screening echocardiography, symptomatic patients repeatedly. In the presence of pulmonary hypertension (PH) unexplained by other diseases, patients underwent ventilation/perfusion lung scan performed to confirm/exclude perfusion defects typical for CTEPH. If PH and perfusion defects persisted despite effective 3-month anticoagulation therapy, patients underwent right heart catheterization to confirm/exclude CTEPH. RESULTS: The study confirmed a higher incidence of CTEPH after splenectomy compared to published data, the 5-year cumulative incidence was 3.2 %. Other detected clinical risk factors did not affect the incidence of thromboembolism/CTEPH after splenectomy. In our study, the strongest factor in terms of the incidence of thromboembolism/CTEPH after splenectomy was the presence of a thrombophilia detected before the screening echocardiography. Tested haemocoagulation and biochemical parameters in small patient subgroup had no impact on the incidence of thromboembolism/CTEPH - however, the limiting factor was a small patient subgroup. CONCLUSION: The results of the study suggest that the incidence of thromboembolism after splenectomy was consistent with the present data, but the incidence of CTEPH after splenectomy was significantly higher. This suggests that post-splenectomy condition may be an independent risk factor for CTEPH and may imply different management of these patients in the future (Tab. 5, Ref. 18).

Central versus Peripheral CTEPH-Clinical and Hemodynamic Specifications.

Background and Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is a chronic progressive disease, resulting from persistent arterial obstruction combined with small-vessel remodeling. Central and peripheral CTEPH are distinguished, according to the dominant lesion's location. This is important for surgical or percutaneous interventional assessment or for medical treatment. Material and Methods: Eighty-one patients (51 male/30 female) with confirmed CTEPH were analyzed, while the CENTRAL type included 51 patients (63%) and the PERIPHERAL type 30 patients (37%). Results: A significant difference in CENTRAL type vs. PERIPHERAL type was determined in gender (male 72.5% vs. 46.7%; p = 0.0198). No difference was found in age, functional status, or echocardiographic parameters. Invasive hemodynamic parameters showed a significant difference in mean pulmonary arterial pressure (46 vs. 58 mmHg; p = 0.0002), transpulmonary gradient (34 vs. 47 mmHg; p = 0.0005), and cardiac index (2.04 vs. 2.5 L.min.m2; p = 0.02) but not in pulmonary vascular resistance. Risk factors showed a significant difference only in acute pulmonary embolism (93.8% vs. 60%; p = 0.0002) and malignancy (2% vs. 13.3%; p = 0.0426). Conclusions: Our study showed hemodynamic differences between CENTRAL type vs. PERIPHERAL type CTEPH with a worse hemodynamic picture in CENTRAL form. This may indicate a different pathophysiological response and/or possible additional influences contributing especially to the peripheral pulmonary bed affection.

Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension-A Case Study Series.

Background. Congenital absence of the portal vein (CAPV) is an extremely rare malformation that is caused by aberrant venous development during embryogenesis and is usually associated with congenital portosystemic shunts (CPSS). This hemodynamic allows mesenteric blood to bypass the liver metabolism and causes an imbalance between vasodilators and vasoconstrictors in the pulmonary circulation, which, again, might lead to the development of secondary portopulmonary hypertension (PoPH). Establishing the exact morphology of the splanchnic venous system is important when evaluating possible therapeutic options (differentiating type I and II CAPV), because some variants enable the closure of the shunt, and this represents a potential cure for pulmonary arterial hypertension (PAH). Once PoPH is diagnosed, complex care in a specialized expert centre is necessary. If possible, CPSS closure is recommended. For long-term successful patient management, specific targeted PAH therapy administration is crucial. Significant morbidity and mortality in these patients may result not only from PAH itself but also due to specific PoPH complications, such as compression of the left main coronary artery by pulmonary artery aneurysm. Case Report. We report on two patients with PoPH due to CAPV and CPSS (without any liver disease) who presented as severe PAH and who, before admission to our expert centre, were misdiagnosed as idiopathic PAH. The case reports also represent our experience with respect to the long-term follow-up and PAH-specific medical treatment of these patients, as well as the possible (even fatal) complications of these rare and complex patients.

[What may cause diabetes]. / Co môze zaprícinit diabetes.

UNLABELLED: The case study describes a case of 49-year-old man with morbid obesity since childhood (BMI 40 kg/m2), arterial hypertension (approx. since aged 15, treated since 2004), dyslipidemia (since 2006), type 2 diabetes mellitus (since 2006, on insulin therapy since 2008) and smoking (until 2011, 20 cigarettes a day). TREATMENT: 16 types of medication, 8 for hypertension, statin, therapy for diabetes, aspirin, allopurinol. In 2010 (when aged 45) hospitalized in our clinic with dyspnoea and chest pain with a high pressure reading of 180/110 mm Hg (identified symptoms of heart failure with LV ejection fraction of 33 %, in NYHA II functional class, echocardiographically: left atrium: 46 mm, left ventricular chamber size in diastole: 70 mm, interventricular septum: 12 mm, septal hypokinesis, Doppler ultrasonography of lower limb arteries (calcification, diffuse atherosclerotic changes, absent stenosis), CT coronary angiography (significant stenosis of the left coronary artery). Treatment started with 40 mg oral dose of furosemide daily. In May 2011 he was hospitalized with an acute coronary syndrome: acute NSTEMI of the inferior wall (coronarography: 2-vascular problems, implemented PKI, implanted DES - ramus circumflexus, paroxysmal atrial fibrillation, NYHA III functional class, left ventricular ejection fraction: 30 %, pulmonary hypertension). In 2012 renal denervation for resistant hypertension was carried out, carotid stent implanted for stenosis of the carotid artery, presence of diabetic nephropathy (KDOQI stage 3, GF 40 ml/min). In August 2014 admitted to our clinic with pulmonary oedema, cardiogenic shock, acute ischemia of the right calf with peripheral embolisation, presence of atrial flutter, impairment of renal parameters, echocardiographically: left atrium: 55 mm, left ventricle size: 75 mm, akinesis of the septum and posterior wall, occlusion of the right leg arteries (given the patients serious state angio-surgical intervention was contraindicated, vitally indicated leg amputation considered), the patient died after 4 days of hospitalization in an intensive care unit after unsuccessful treatment. A combination of diabetes, hypertension and ischemic heart disease is frequent and prognostically serious. Diabetes increases cardiovascular morbidity and mortality and therefore we should check for diabetes in all cardiovascular patients.