Extra-Cavity Image Registration via the Aortic Root During Left Ventricular Mapping and Ablation.

BACKGROUND: Computed tomography (CT) image integration is of limited use in left ventricular (LV) ablation due to inadequate accuracy of registration. The current study aimed to investigate the accuracy and feasibility of extra-cavity LV image registration via the coronary cusp. METHODS: Consecutive patients were enrolled as the validation group (n = 41) and feasibility group (n = 48). After extra-cavity registration via the aortic root, the LV anatomy derived from CT image was activated and moved into real space. Accuracy of LV anatomy via this registration method was verified by intracardiac echocardiography reconstruction in the validation group and tested further in the feasibility group via measuring the location differences (<3 mm) and volume difference (<8 mL). RESULTS: In validation group, the LV volume of CT image and ICE map were comparable (113.6 ± 15.5 mL vs. 109.0 ± 15.3 mL, P =.27), and the location difference was 3.1 ± 1.1 mm at LV summit, 1.8 ± 0.9 mm at the free wall, and 1.8 ± 0.7 mm at the LV apex. There was a mean of 2.9 ± 1.2 mm and 3.0 ± 1.0 mm length difference in anterior PM and posterior PM, the position difference of the PM's base was 2.8 ± 0.9 mm for anterior PM and 2.2 ± 0.9 mm for posterior PM. In feasibility group, the distance differences of LV summit, LV septum, LV apex, and LV free averaged 1.8 ± 0.8 mm, 1.5 ± 0.7 mm, 1.4 ± 0.6 mm, 1.3 ± 0.7 mm, respectively. Compared with validation group, acute success (100% vs. 96.5%, P =.51), complications rate (4.9% vs. 2.0%, P = 0.59) and fluoroscopic time (1.6 ± 1.1 vs. 1.9 ± 1.6 minutes, P =.30) exhibited no significant difference, but was significantly reduced with procedure time (74.5 ± 8.1 vs. 61.2 ± 9.5 minutes, P <.001) with CT image registration only. CONCLUSION: LV mapping and ablation could be successfully achieved by extra-cavity registration via coronary cusp without needing positions within LV beforehand.

Pediatric restrictive cardiomyopathy: a case report.

Restrictive cardiomyopathy (RCM) is a rare childhood cardiomyopathy that is a challenging diagnostic problem for clinicians. We describe a case of an 8-year-old girl with a 2-year history of shortness of breath on exertion. Electrocardiogram and echocardiography showed biatrial enlargement, while cardiac magnetic resonance showed biatrial dilation and normal pericardial thickness. Left and right heart catheterization revealed a left ventricular (LV) end-diastolic pressure (EDP) of 20 mmHg, right ventricular (RV) EDP of 13 mmHg, and pulmonary arterial systolic pressure of 51 mmHg. LV and RV pressure traces showed that LV and RV pressures moved concordantly with respiration, and that the systolic area index was 0.98. Cardiac catheterization data were therefore supportive of RCM. Next-generation sequencing identified a heterozygous variant of the troponin I gene (TNNI3; c.574C>T). Combining these findings led to a diagnosis of RCM. The patient's parents chose conservative treatment, but at the 12-month follow-up she died of worsening heart failure and cerebral infarction. This case emphasizes the need for cardiac catheterization and genetic testing in RCM, and suggests that anticoagulants should be recommended to reduce the risk of thromboembolic events.

Pulmonary Hypertension in a Patient With Kartagener's Syndrome and a Novel Homozygous Nonsense Mutation in CCDC40 Gene: A Case Report.

Kartagener's syndrome is a subgroup of primary ciliary dyskinesia (PCD), a genetically heterogeneous condition characterised by sinusitis, bronchiectasis, and situs in versus. Genetic testing has importance for their diagnosis. Here, we report a chinese patient with Kartagener's syndrome. Transthoracic echocardiography showed severely elevated right ventricular systolic pressure. Right heart catheterisation demonstrated a pre-capillary pulmonary hypertension. Whole-exome sequencing indicated that she had a novel homozygous nonsense mutation, c.2845C > T, p.Gln949*, in exon 18 of CCDC40 and a heterozygotic mutation, c.73G > A, p.Ala25Thr, in exon 1 of DNAH11. She was diagnosed as Kartagener's syndrome with pulmonary hypertension. Her symptoms improved significantly by treatment of antibiotics, expectorant drugs, bronchodilators, and oxygen therapy treatment. Our findings extend the mutation spectrum of CCDC40 gene related Kartagener's syndrome, which is very important for gene diagnosis of the disease.

Cierre percutáneo de una enorme fístula arteriovenosa pulmonar / Transcatheter closure of a huge pulmonary arteriovenous fistula

No disponible

Prevalence of Congenital Heart Disease Amongst Schoolchildren in Southwest China.

OBJECTIVE: To investigate the prevalence and risk factors of congenital heart disease in Yunnan, China which has diverse ethnic groups. METHODS: This cross-sectional study enrolled 244,023 children from 2010 to 2015. To diagnose CHD, a conventional physical examination was used to screen suspicious cases, which were further confirmed by echocardiography. RESULTS: A total of 1695 children were diagnosed with CHD. The estimated prevalence was 6.94%. Atrial septal defects were the most common cardiac abnormalities. A higher prevalence of CHD was observed with preterm birth, low birth weight, maternal age ≥35 years, and high-altitude regions. The prevalence also showed differences between diverse ethnic groups. CONCLUSIONS: The prevalence of CHD in China may have ethnic differences.

Pulmonary arterial hypertension in HIV infection: a concise review.

Pulmonary arterial hypertension (PAH) is an infrequent but nevertheless serious life threatening severe complication of human immunodeficiency virus (HIV) infection. In today's era of antiretroviral therapy (ART), the mortality of HIV patients has greatly reduced due to improved immune function and fewer opportunistic infections. However, these patients have an increased incidence of PAH. In this review, we will mainly discuss HIV-related pulmonary arterial hypertension (HRPH) in terms of the epidemiology, pathogenesis, clinical characteristics and treatment.

[Retrospective analysis of patients with thrombocytopenia after patent ductus arteriosus interventional occlusion].

OBJECTIVE: To explore the risk factors of patent ductus arteriosus (PDA) patients with thrombocytopenia after PDA interventional occlusion. METHODS: Thrombocytopenia occurred in 14 out of 350 patients underwent PDA occlusion. Age, gender, body weight, PDA size, occluder size, mean pulmonary arterial pressure, the dose of heparin, the manufacturer of occluder, residual shunt after operation were analyzed. The recovery time of different grades of thrombocytopenia was observed. RESULTS: Multivariate logistic regression showed that the PDA size (OR = 2.238, P < 0.05), the dose of heparin (OR = 3.247, P < 0.05), residual shunt after operation (OR = 1.912, P < 0.01) were the independent risk factors of thrombocytopenia after PDA occlusion. The recovery time of mild thrombocytopenia was (7 ± 2) days without treatment. The recovery time of moderate thrombocytopenia was (12 ± 4) days with glucocorticoids treatment. The recovery time of severe thrombocytopenia was (21 ± 7) days with platelet transfusion. CONCLUSIONS: The occluder size, dose of heparin, residual shunt are the independent risk factors of thrombocytopenia after PDA interventional occlusion. Recover time of thrombocytopenia after PDA interventional occlusion is closely related to the severity of thrombocytopenia.

The changes of serum angiotensin-converting enzyme 2 in patients with pulmonary arterial hypertension due to congenital heart disease.

BACKGROUND: Angiotensin-converting enzyme 2 (ACE2), a primary component of the vasoprotective axis in the renin-angiotensin system (RAS), has recently been found to have regulatory actions in hypoxic pulmonary hypertension and monocrotaline-induced pulmonary hypertension. We explored the hypothesis that the level of ACE2 protein contents may be decreased in patients with pulmonary arterial hypertension (PAH) due to congenital heart disease (CHD). OBJECTIVE: We observed the serum ACE2 protein contents in patients with PAH due to CHD (CHD-PAH), and investigated their correlation with mean pulmonary arterial pressure (mPAP). METHODS: One hundred and four patients with CHD and 33 normal control patients (group A) were involved in the research. The patients with CHD were divided into 55 cases of nonpulmonary hypertension (group B), 25 cases of mild to moderate pulmonary hypertension (group C) and 24 cases of severe pulmonary hypertension (group D). The serum level of ACE2 protein contents were detected by enzyme-linked immunosorbent assay (ELISA), and the relationship between these contents and mPAP were analyzed. RESULTS: ACE2 protein contents significantly declined as mPAP increased. The mPAP was negatively correlated with the level of ACE2 protein contents. CONCLUSIONS: These results demonstrated that ACE2 may play an important regulatory role in CHD-PAH.

Application of echocardiography in resynchronization treatment of heart failure patients.

OBJECTIVE: To review the updated research progress about the application of echocardiography in resynchronization treatment of chronic heart failure patients. DATA SOURCES: The data used in this review were from PubMed, published in English and using the key terms "heart failure", "echocardiography" and "cardiac resynchronization therapy". STUDY SELECTION: Relevant articles were reviewed and selected to address the stated purpose. RESULTS: Increasing numbers of studies have suggested the importance of echocardiography in resynchronization treatment of chronic heart failure patients. Echocardiography can evaluate atrioventricular, inter- and intra-ventricular mechanical dyssynchrony before cardiac resynchronization therapy (CRT), as a guidance to assess the optimal left ventricular (LV) pacing location, optimize the atrioventricular and interventricular delays and predict response to CRT. CONCLUSIONS: Echocardiography is both non invasive and easily repeatable, and plays a crucial role in appraisal of heart synchronism, instruction of actuator placement, optimization of the device procedure, and prediction of the response to CRT.