Role of cardiac magnetic resonance imaging in myocarditis patients with biopsy negative: a retrospective case series study / 中华心血管病杂志

Objective: To analyze the cardiac magnetic resonance (CMR) imaging feature of clinically diagnosed myocarditis patients with negative endocardial biopsy (EMB) results, and to further demonstrate the diagnostic value of CMR in these patients. Methods: This was a retrospective case series study. Fourteen patients, who were clinically diagnosed as myocarditis according to 2013 European Society of Cardiology (ESC) clinical diagnostic criteria for myocarditis, but with negative EMB results, were enrolled. All patients underwent CMR examinations. The morphological, functional and histological changes of the heart were assessed based on black blood sequence, cine sequence, T2W-STIR sequence and contrast agent late gadolinium enhancement,(LGE). Results: There were 10 males and 4 females in this cohort, the age was (25.6±13.2) years. The interval between symptom onset and CMR was 21 (13, 60) days, and the interval between symptom onset and EMB was 19 (9, 40) days. There were 13 patients with abnormal CMR results including myocardial oedema, fibrosis, decreased ejection fraction, pericardial effusion or increased cardiac chamber dimension. Nine out of 14 patients had CMR morphological and/or functional abnormalities, including 1 case of left atrium enlargement, 1 case of left ventricle enlargement, 3 cases of right ventricle enlargement, 4 cases of increased left ventricular end diastolic volume index. Left ventricular ejection fraction was<50% in three cases, right ventricular ejection fraction was<40% in 5 cases, and pericardial effusion depth>3 mm was detected in 3 cases. Of the 14 patients, 11 had histological changes, of which 6 had T2 ratio≥2. Among the 10 patients (10/14) with positive LGE, the most common patterns were subepicardial LGE of the lateral wall and/or midwall LGE of the septum (n=9); 2 cases showed extensively subendocardial LGE of the left ventricular wall. No LGE involved in the right ventricular wall in the whole cohort. Conclusion: CMR plays a complementary role in the diagnosis of myocarditis in clinically diagnosed myocarditis patients with negative EMB findings.

Feasibility and safety of right ventricular endomyocardial biopsy via the right internal jugular vein approach / 中华心血管病杂志

Objective: To evaluate the feasibility and safety of right ventricular endomyocardial biopsy (EMB) via the right internal jugular vein approach. Methods: It was a retrospective and descriptive study. A total of 272 patients, who underwent right ventricular EMB from December 2014 to June 2020 in Fuwai Hospital and Peking Union Medical College Hospital were enrolled. The preliminary diagnosis included suspected myocarditis, myocardiopathy, unexplained heart failure etc after exclusion of coronary heart disease. Clinical characteristics including age, sex, height, weight, NYHA functional class, NT-proBNP, chest radiography, echocardiography, and hemodynamics parameters were collected at baseline. EMB was performed via right internal jugular vein approach under the biplane fluoroscopic guidance. Success rate was calculated in this study. Complications related to operation were recorded according the following definitions. Major complications included death, urgent cardiac surgery, advanced cardiac life support, pericardiocentesis in cardiac tamponade, permanent complete atrioventricular block requiring permanent pacing etc. Minor complications included pericardial effusion without pericardiocentesis, temporary (lasting less than 24 hours) or permanent right bundle-branch block, temporary Mobitz type Ⅱ atrioventricular block (AV block) with AV conduction 2∶1 requiring medical treatment with atropine, or additive temporary pacing, non-sustained ventricular tachycardia with long runs of more than 10 ventricular complexes, and an episode of atrial fibrillation lasting less than 12 hours or cardioversion of atrial fibrillation. Other complications included tricuspid anterior chorda rupture and new onset tricuspid regurgitation after EMB. Results: In this study, right ventricular EMB were performed successfully in 270 patients, the total success rate was 99.3% (270/272), and EMB were failed in 2 (0.7%) patients. Age of the enrolled patients was (42.7±16.9) years, and there were 164 (60.3%) males. Major complication including cardiac tamponade requiring pericardiocentesis occurred in 2 (0.7%) patients. Minor complications such as small amount pericardial effusion occurred in 18 (6.6%) patients, tricuspid anterior chorda rupture occurred in 1 (0.4%) patient. No patient died, or requiring permanent pacing, or requiring emergency cardiac surgery. The complication rate was 9.3% (13/140), 7.8% (7/90), and 2.4% (1/42) in operators with 1, 2, and 3 years' experience. Conclusions: EMB via the right jugular vein approach under fluoroscopic guidance is a simple, safe and feasible procedure. The complication rates decrease significantly with increasing operator experience.

Aerosolized iloprost therapy for pulmonary hypertensive crisis in 4 patients with idiopathic pulmonary arterial hypertension / 中华心血管病杂志

<p><b>OBJECTIVE</b>To summary the efficacy and safety of aerosolized iloprost in patients with pulmonary hypertensive crisis.</p><p><b>METHODS</b>On the basis of conventional therapy, aerosolized iloprost (10 µg per time for 10 - 15 min in 2 hours interval, 8 times per day) was administered to four patients with idiopathic pulmonary arterial hypertension and pulmonary hypertensive crisis. Blood pressure, heart rate, systemic artery oxygen saturation, systolic pulmonary arterial pressure (sPAP) measured by echocardiography and the adverse events were analyzed.</p><p><b>RESULTS</b>After aerosolized iloprost therapy, sPAP was significantly decreased and systemic artery oxygen saturation was improved. Adverse events (nausea, vomiting, diarrhea, dry cough) were observed in two patients, and the iloprost use was stopped in one patient due to severe vomiting and diarrhea.</p><p><b>CONCLUSION</b>Aerosolized iloprost could significantly reduce the sPAP and improve the systemic artery oxygen saturation in patients with pulmonary hypertension crisis.</p>

Clinical efficacy of intravenous L-carnitine in patients with right-sided heart failure induced by pulmonary arterial hypertension / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the efficacy of L-carnitine in patients with right-sided heart failure induced by pulmonary arterial hypertension (PAH).</p><p><b>METHODS</b>A total of 66 pulmonary arterial hypertension patients (14 idiopathic, 36 congenital heart disease associated and 16 connective-tissue disease associated PAH, WHO heart functional class III, n = 38 or IV, n = 28) were enrolled in this study and divided into control group (26 cases) and L-carnitine group (40 cases). All patients received conventional treatment according to guideline for treatment of right-sided heart failure. The patients in L-carnitine group received 5 g/d L-carnitine intravenously for seven days. Six-minute walking distance, WHO heart functional class, physical examination, and serum markers were evaluated at baseline and 7 days after enrollment.</p><p><b>RESULTS</b>Compared to the baseline, six-minute walking distance was significantly increased (75 m vs. 45 m, P < 0.05), WHO heart functional class significantly improved (improved 2 classes in 16 patients, improved 1 class in 13, no improved in 6, worsen in 5 vs. 3, 8, 9, 6 respectively in the control, P = 0.04), BNP level significant decreased (58.16 ng/L vs. 33.29 ng/L, P = 0.01) and systolic blood pressure significantly increased [8.1 mm Hg vs. 2.4 mm Hg (1 mm Hg = 0.133 kPa), P = 0.03] in L-carnitine group compared with those in control group. No patient was withdrawn from this study for safety reasons.</p><p><b>CONCLUSIONS</b>L-carnitine could improve short-term exercise capacity and WHO heart functional class in right-sided heart failure patients induced by PAH.</p>

Diagnosis value of electrocardiogram in patients with pulmonary artery hypertension / 中华心血管病杂志

<p><b>OBJECTIVES</b>To analyze the diagnostic value of electrocardiogram (ECG) in patients with pulmonary artery hypertension (PAH) confirmed by right-heart catheterization (RHC).</p><p><b>METHODS</b>A total of 64 patients with suspected PAH [sPAP > or = 36 mm Hg (1 mm Hg = 0.133 kPa) estimated by echocardiography] were enrolled in this study. All patients were examined by 12-lead ECG within half an hour before RHC.</p><p><b>RESULTS</b>PAH was excluded in 26 patients and confirmed in 38 patients. ECG analysis showed that S amplitude > 0.21 mV in lead I, QRS axis > 87 degrees , R(V1) + S(V5) > 0.76 mV were good parameters for diagnosing PAH with sensibility and specificity of 89%, 81%; 86%, 92%; 84%, 83%, respectively. QRS axis was positively correlated with mean pulmonary artery pressure (mPAP) (r = 0.75, P < 0.001), R(V1) + S(V5) was positively correlated with pulmonary vascular resistance (PVR) (r = 0.74, P < 0.001), R(V1) + S(V5) and S amplitude in lead I was negatively correlated with cardiac index (CI) (r = -0.62, P < 0.001).</p><p><b>CONCLUSION</b>ECG combined with echocardiography are adequate screening tools to rule out the presence of PAH. QRS axis, R(V1) + S(V5) and S amplitude in lead I were significantly correlated with hemodynamic parameters derived from RHC in PAH patients.</p>

Feasibility and safety of right heart catheterization and pulmonary angiography through the antebrachium veins / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the feasibility and safety of right heart catheterization through the antebrachium veins.</p><p><b>METHODS</b>A total of 68 consecutive patients suspected with pulmonary vascular diseases underwent standard right heart catheterization and pulmonary angiography through the antebrachium veins were enrolled in this multicenter, cross-sectional study.</p><p><b>RESULTS</b>The rate of successfully inserting the sheath into antebrachium veins was as high as 97.1% (66/68) and the rate of successfully performing right heart catheterization or pulmonary angiography through vascular access of antebrachium veins was 91.2% (62/68). The reasons of unsuccessful inserting the catheter to the right heart were due to the abnormality of antebrachium veins (2 cases) or stenosis of subclavian vein (3 cases) or unsatisfactory engorging of antebrachium veins since the history of drug injection (1 case). Haemorrhage of branch of axillary vein was the only adverse event occurred in one patient.</p><p><b>CONCLUSION</b>It is a safe, convenient and well-tolerant option to perform right heart catheterization and pulmonary angiography through the vascular access of antebrachium veins.</p>

Pulmonary function testing in Chinese patients with pulmonary arterial hypertension / 中华心血管病杂志

<p><b>OBJECTIVE</b>To determine the diagnostic value of pulmonary function testing in Chinese patients with known pulmonary arterial hypertension (PAH) without history of lung/heart valve diseases.</p><p><b>METHODS</b>Pulmonary function testing was performed in 41 PAH patients diagnosed by right heart catheterization and in 17 healthy controls.</p><p><b>RESULTS</b>Normal pulmonary function testing results were found in 5 PAH patients (12.2%). Total lung capacity, vital capacity and FEV1 were significantly decreased in PAH patients [(80.27 +/- 11.46)% vs. (94.24 +/- 6.82)%; (79.09 +/- 14.89)% vs. (97.35 +/- 9.51)%; (75.40 +/- 16.58)% vs. (95.12 +/- 12.01)%, respectively, all P < 0.001], the ratio of residual volume/total lung capacity was significantly increased [(117.67 +/- 25.73)% vs. (93.39 +/- 10.87)%, P < 0.001]; FEV1/FVC and maximal expiratory flow of 25% to 75% tended to be lower (-6.0% and -19.4%, P = 0.21 and 0.09) while DLCO and DLCO/VA were significantly decreased by 36.6% and 29.8% (P < 0.001) compared with healthy controls.</p><p><b>CONCLUSIONS</b>Increased peripheral airway obstruction and normal lung resistance were found in these PAH patients. Normal pulmonary function testing results could not rule out the diagnosis of PAH.</p>

Hemodynamic variables and clinical features correlated with serum uric acid in patients with pulmonary arterial hypertension / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Serum uric acid (UA), the final product of purine degradation, has been proposed to be a marker for the severity and a possible predictor of mortality in patients with pulmonary arterial hypertension (PAH). The objectives of this study were to elucidate whether serum UA level correlates with the clinical features and the hemodynamic variables in Chinese patients with PAH and to compare the difference of the correlates in patients associated with different etiologies.</p><p><b>METHODS</b>Serum UA was assessed in 228 patients with three types of PAH (idiopathic PAH (IPAH), congenital heart disease related PAH (CHD-PAH) and connective tissue disease related PAH (CTD-PAH)) together with other clinical features. After the individualized treatment for at least 6 months, the UA levels and clinical features were re-evaluated in 88 patients.</p><p><b>RESULTS</b>Serum UA was significantly elevated in patients with PAH compared with age-matched control subjects ((350.40 +/- 108.73) micromol/L vs (266.91 +/- 81.38) micromol/L), P < 0.001). Serum UA negatively correlated with cardiac output and mixed venous saturation (SvO(2)) in all three types of PAH (all P < 0.05), positively correlated with the size of right ventricle in IPAH (P = 0.002) and CTD-PAH (P = 0.013) patients and with pulmonary vascular resistance just in CTD-PAH patients (P = 0.001). Serum UA significantly decreased from (365.80 +/- 120.46) micromol/L to (333.67 +/- 117.56) micromol/L in 88 patients (P = 0.006) with vasodilator therapy for at least 6 months, accompanied with a reduction in pulmonary vascular resistance from (15.13 +/- 6.96) Woods unit to (12.00 +/- 5.04) Woods unit (P = 0.001) and an increase in cardiac output from (2.63 +/- 0.98) L/min to (3.08 +/- 1.04) L/min (P = 0.005).</p><p><b>CONCLUSIONS</b>Serum UA increases in proportion to the clinical severity of all the three types of PAH, especially the CTD-PAH had a stronger correlations compared with IPAH and CHD-PAH. The serum UA levels also could partly reflect the response to the treatment in patients with PAH.</p>

Polymorphisms of angiotensin-converting enzyme 2 gene associated with magnitude of left ventricular hypertrophy in male patients with hypertrophic cardiomyopathy / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Even carrying an identical gene mutation, inter- and intra-family variations have been noticed worldwide in the presence and the severity of left ventricular hypertrophy and sudden death in patients with hypertrophic cardiomyopathy (HCM). Modifier genes may contribute to the diversity. Angiotensin-converting enzyme 2 (ACE2) gene has been established to be associated with parameters of left ventricular hypertrophy in community based male subjects. The objective of the present study was to investigate the association of ACE2 gene polymorphisms with the phenotype of HCM.</p><p><b>METHODS</b>A total of 261 consecutive HCM patients and 609 healthy controls were enrolled into this study. The polymorphism of rs2106809 and rs6632677 were genotyped by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) and confirmed by sequencing. Logistic regression model and multivariate analysis were used to determine the odds ratio (OR) and 95% confidence intervals (CI) of variations of ACE2 for HCM.</p><p><b>RESULTS</b>The T allele of rs2106809 and C allele of rs6632677 conferred increasing risk for HCM (OR 1.34, 95% CI 1.01 - 1.77, P = 0.04; OR 1.11, 95% CI 1.03 - 1.21, P = 0.002, respectively), and the 2 single nucleotide polymorphisms (SNPs) were in strong linkage disequilibrium (LD), the TC haplotype was independently associated with a higher OR for HCM (OR = 1.59, 95% CI 1.21 - 1.87) after adjusted for conventional risk factors. And the risk alleles were associated with thicker interventricular septal thickness of HCM ((20.0 +/- 6.3) mm vs (17.9 +/- 5.5) mm, P = 0.03 and (21.3 +/- 5.9) mm vs (17.9 +/- 5.8) mm, P = 0.04, respectively). No association was found between the two polymorphisms with female patients with HCM.</p><p><b>CONCLUSION</b>Minor alleles of ACE2 gene might be the genetic modifier for the magnitude of left ventricular hypertrophy in male patients with HCM.</p>