Multidisciplinary Cardiovascular Annals
Volume:14 Issue: 1, Jan 2023

 Given the significant morbidity and mortality of juvenile pulmonary arterial hypertension (PAH), understanding the clinical and paraclinical findings of this condition can aid in the early diagnosis and identification of helpful factors in its treatment.

 This study aimed to evaluate the clinical and paraclinical findings of idiopathic pulmonary arterial hypertension (iPAH) in children.

 This cross-sectional study was carried out on 20 children with iPAH who were assessed in terms of pulmonary hypertension or hospitalized at the cardiac ward of Imam Reza hospital, Mashhad, Iran, during January 2018 to October 2001. The clinical data, chest X-ray (CXR), electrocardiographic, echocardiographic, cardiac catheterization findings, and mortality results were extracted from the medical records and recorded in a researcher-made questionnaire.

 The current study’s findings revealed a higher prevalence of iPAH in females (70%) than men, as well as in term neonates compared to preterm newborns. The most common clinical findings were active dyspnea, heart murmur, and cyanosis. Prominent pulmonary conus, right atrial enlargement (RAE), and right ventricular hypertrophy (RVH) were the most common CXR findings. The right axis deviation, RVH, and RAE were the most common abnormal findings of electrocardiography, respectively. Based on echocardiographic findings, dilated right atrium and dilated right ventricle were reported in all subjects. The mean of tricuspid regurgitation pressure gradient (TRPG) and peak systolic pressure gradient (PSPG) were 76.33 ± 22.8 and 44.2 ± 13.18 mmHg, respectively. The mean ratios of systolic pulmonary artery pressure (PAP), diastolic PAP, and mean pulmonary artery pressure (mPAP) to aortic systolic pressure were estimated at 0.92, 0.78, and 0.88, respectively. The results confirmed a significant relationship between mPAP and consanguinity (P = 0.03). In addition, the findings demonstrated a correlation between echocardiographic findings (i.e., TRPG and PSPG) and systolic PAP, diastolic PAP, and mPAP (P < 0.05). In total, 56.3% of the patients expired, most of whom (37.5%) were the younger children.

 The findings suggest that IPAH should be considered as a possible diagnosis in various clinical manifestations, including respiratory distress, hepatomegaly, ascites and even neurological symptoms. Since there are no specific symptoms, therefore a set of history, physical examination, laboratory findings, and clinical suspicion should be taken into consideration.

 Endourologic or percutaneous interventional treatments in genitourinary (GU) is a growing field of science with the advent of improved imaging techniques and smaller catheters. Hereby, we report case series of endovascular GU angioembolization procedures performed in our center.

 In this study, we report cases who underwent successful angioembolization. A total of 76 cases of bladder transitionary cell carcinoma cells, 8 cases of bladder and renal arteriovenous malformation (AVMs), 33 cases with hematuria after percutaneous nephrolithotomy (PCNL), 29 cases of renal angio-myolipoma, 3 benign prostatic hyperplasia (BPH) cases high risk for surgery and 5 cases of hematuria in the setting of acute renal trauma had undergone GU endovascular treatment.

 The success rate for angioembolization of bladder transitionary carcinoma cell was 100% but in 2 cases hematuria continued due to another GU source. Coil embolization of bladder and renal AVMs and renal angio-myolipoma were successful in all cases. Cessation of hematuria after PCNL was successful in all cases except one. Coli embolization of BPH and hematuria due to renal trauma were ceased in all cases. Complications included easily controllable hematoma in 3 cases. Contrast induced nephropathy and fever occurred in 12% and 20%, respectively. Pain duration was within 4 - 10 days after embolization of AVM or tumor. Embolization could be done in high output priapism and varicocel but we did not perform these procedures because of no referral.

 Coil embolization of GU system is quite safe and approved method and could be used with high success rate and low failure rate.

Acute and severe rises in systemic blood pressure during or after cardiac surgery may be life-threatening and result in end-organ injury. This case is the first report of spontaneous left atrial appendage rupture following a hypertensive crisis in cardiac surgery. Ethical approval for this report was obtained from the Ethics Committee.

 Congenital malformation of mitral valve could encounter in isolation or with other congenital heart diseases.

 Here, we report a case of congenital hypoplastic anterior mitral valve leaflet (AMVL).

 A 30-year-old male with a history of diabetes mellitus was referred with pleuritic chest pain since the previous day. He was admitted with impression of pericarditis. Trans thoracic echocardiography showed no pericardial effusion with incidental finding of lipomatous change of mitral valve annulus and hypoplastic AMVL with mild to moderate mitral regurgitation.

 Cardiac magnetic resonance imaging showed thickening and fibrosis replacement of intervalvular fibrosa which extended to AMVL and resulted in restricted mitral valve annulus (12.4 mm) with subsequent restricted MV opening without significant mitral stenosis or regurgitation. These data were in favor of congenital hypoplastic MV annulus.

 Hypoplastic AMVL with restricted mitral valve annulus is a rare cause of mitral valve malformation and could be well tolerated since adulthood with good prognosis.

 Aberrant right subclavian artery (ARSA) aneurysm repair is usually performed surgically.

 We report the case of an endovascular exclusion of ARSA aneurysm using a degressive covered endograft via axillary access. This technique can avoid classical complications of an open surgical repair and the complexity of hybrid techniques, especially in patients of advanced age with multiple comorbidities.

Infectious endocarditis is a rare infectious disease of the cardiac endothelium, but blood culture-negative endocarditis (BCNE) should be considered.

This study aims to determine the prevalence of possible infectious agents of BCNE.

Real-time PCR and PCR sequencing were used to identify Coxiella burnetii, Tropheryma whipplei, Brucella spp., Legionella spp., and Bartonella spp. 16srRNA sequencing was used for the samples with negative results of PCR sequencing and real-time PCR.

Among the 59 positive results, the rates of Bartonella spp., Legionella spp., and Brucella spp. were 8.5%, 5.1%, and 3.4%, respectively. C. burnetii and T. whipplei were not detected. 16srRNA sequencing revealed 1 Stenotrophomonas maltophilia, 1 Aeribacillus spp. and 1 uncultured bacterium.

The rate of some bacteria that are difficult to detect with routine laboratory tests was high in our study, and it seems that hospitals should use dedicated technology to detect BCNE pathogens.