Etiological classification and manifestation of pulmonary artery dissection: A literature review and case analysis.

Background: Pulmonary artery dissection (PAD) is an uncommon condition associated with high mortality rates. To date, a comprehensive examination of various underlying causes of PAD remains unexplored. Methods: We used the search words "pulmonary artery dissection," "pulmonary artery dilatation," and "pulmonary artery rupture" in the public database, and examined the medical records of PAD patients treated in our hospital. Data on demographics, aetiology, PAD locations, histopathology, treatments, and outcomes, were collected. Results: A total of 145 patients with PAD (135 cases from the literature and 10 cases from our hospital) were analysed. PAD aetiology was categorized into four groups: congenital heart diseases (CHD) associated with pulmonary arterial hypertension (PAH), non-CHD associated with pulmonary hypertension (PH), aortic dissection-related, and miscellaneous causes. The most frequent cause, accounting for 32.4 % of PAD cases, was congenital heart disease, followed by idiopathic PAH (13 %) and chronic obstructive pulmonary disease (6 %). Patients with CHD were typically younger at the time of PAD diagnosis (median age: 35 years old) when compared with those suffering from aortic dissection, PH-associated conditions, or other causes (median age: 45, 55, and 56 years old, respectively). Imaging of the pulmonary artery proved effective in diagnosing PAD. The outcomes were generally poor. 44.7 % (21/47) of patients with CHD associated with PAH and 47.7 % (21/44) of non-CHD PH-associated diseases died during follow-up. Multidisciplinary team consultations are crucial when making decisions on management of PAD. Characteristic PAD histopathology features included thickened intima and hypertrophied media with atheromatous degeneration, disrupted elastic fibres, and lymphocytic infiltration. Conclusions: PAD aetiology can be divided into four main categories, with CHD associated with PAH being the leading cause. Despite the similar histopathology features, clinical manifestations and outcomes vary according to the aetiology.

A case report of a long-term survivor after inadvertent ligation of the left pulmonary artery during intended ductal ligation.

Background: Patent ductus arteriosus (PDA) can close on its own during childhood. Patent ductus arteriosus with left pulmonary artery (LPA) occlusion is rare. Here, we describe possible aetiologies of this condition and treatment strategies based on recent guidelines. Case summary: A 35-year-old man experienced shortness of breath for 20 years. Physical examination revealed pitting oedema, digital clubbing, and bi-phasic murmur along the left sternal border at the 2nd and 3rd intercostal space. Congenital heart disease was suspected. Echocardiography revealed a severely dilated pulmonary trunk and PDA; however, the LPA was not visible. The patient has undergone PDA ligation surgery >30 years ago, which may have caused accidental LPA ligation; however, extreme elevation of pulmonary pressure led to increased difficulties in performing LPA reconstruction and PDA division. Therefore, pulmonary arterial hypertension (PAH) initial combination therapy with parenteral prostanoids was prescribed. The patient's condition improved gradually. Discussion: Inadvertent ligation of the LPA instead of PDA is a rare and usually fatal complication during ductal ligation. Patients who survive this catastrophic complication usually develop the progressive pulmonary vascular disease with increased pulmonary pressure and impaired lung parenchyma resulting in right heart and respiratory failure. Early and regular follow-up with cardiac imaging studies is important to diagnose this complication. Reconstruction of the ligated LPA and PDA late in the disease course is difficult due to the development of pulmonary arterial hypertension. Initial PAH combination therapy may be valuable for relieving the patients' symptoms at that stage. Heart and lung transplantation can also be considered in suitable patients.

The spectrum and prevalence of arrhythmia in different clinical pulmonary hypertension groups in Chinese population.

BACKGROUND: Arrhythmia is not uncommon among pulmonary hypertension (PH) population, and may be associated with disease severity. HYPOTHESIS: To investigate different spectrums and prevalence of arrhythmias in different clinical PH groups in Chinese population. METHODS: Patients diagnosed with PH between April 15, 2019, and August 2, 2021, were enrolled prospectively. The prevalence of different types of arrhythmias in PH patients were calculated. Logistic regression analyses were conducted to determine independent predictors for arrhythmia. RESULTS: One thousand patients were enrolled. The prevalence of any arrhythmia, sinus node dysfunction, sinus tachycardia, atrial fibrillation, atrial flutter, other types of atrial tachycardia, atrioventricular block, and ventricular tachycardia is 44.4%, 12.2%, 15.2%, 8.1%, 4.1%, 10.2%, 7.1%, and 2.5%. Logistic regression analyses revealed that older age and larger right ventricle (odds ratio: 1.111 and 1.095, p < .05) were independently related with higher probability of supraventricular arrhythmia; Complicating with coronary artery disease, larger right ventricle, and increased left ventricular end-diastolic diameter (odds ratio: 19.540, 1.106, and 1.085, p < .05) were independently correlated with sinus node dysfunction/atrioventricular block in patients with pulmonary arterial hypertension. CONCLUSIONS: Nearly half of PH patients experienced at least one type of arrhythmia. The most common seen arrhythmias were supraventricular arrhythmia, sinus tachycardia, and sinus node dysfunction. Older age and larger right ventricle were independently related with higher probability of supraventricular arrhythmia; Complicating with coronary artery disease, larger right ventricle and increased left ventricular end-diastolic diameter were independently correlated with higher probability of sinus node dysfunction/atrioventricular block in patients with pulmonary arterial hypertension.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: State of the art.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complex chronic disease in which pulmonary artery stenosis or obstruction caused by organized thrombus can lead to increased pulmonary artery pressure and pulmonary vascular resistance, ultimately triggering progressive right heart failure and death. Currently, its exact mechanism is not fully understood. Pulmonary endarterectomy (PEA) has immediate effects with low perioperative mortality and satisfactory prognosis in experienced expert centers for CTEPH patients with proximal lesions. Nevertheless, 37% of patients are deemed unsuitable for PEA surgery due to comorbidities and other factors, and nearly half of the operated patients have residual or recurrent pulmonary hypertension. Riociguat is the only approved drug for CTEPH, although its effect is limited. Balloon pulmonary angioplasty (BPA) is a promising alternative treatment for patients with CTEPH. After more than 30 years of development and refinements, emerging evidence has confirmed its role in patients with inoperable CTEPH or residual/recurrent pulmonary hypertension, with acceptable complications and comparable long-term prognosis to PEA. This review summarizes the pathophysiology of CTEPH, BPA history and development, therapeutic principles, indications and contraindications, interventional procedures, imaging modalities, efficacy and prognosis, complications and management, bridging and hybrid therapies, ongoing clinical trials and future prospects.

Two-dimensional speckle tracking echocardiography detected interventricular dyssynchrony predicts exercise capacity and disease severity in pre-capillary pulmonary hypertension.

BACKGROUND: Right ventricular (RV) intraventricular mechanical dyssynchrony detected by two-dimensional speckle tracking echocardiography (2D-STE) has been reported to be correlated with a decrease in RV contractile efficiency in pulmonary hypertension (PH) patients, while little attention has been paid to biventricular dysfunction. Therefore, we aimed to evaluate the predictive value of 2D-STE detected interventricular dyssynchrony for exercise capacity and disease severity in patients with pre-capillary PH (PcPH). METHODS: Conventional transthoracic echocardiography, 2D-STE and cardiopulmonary exercise tests (CPETs) were performed in all participants. Intra- and interventricular dyssynchrony were calculated as the standard deviation (SD) of the time intervals corrected for heart rate between QRS onset and peak longitudinal strain. Multivariate linear regression analyses were performed to identify independent predictors of peak oxygen consumption (PVO2) during the CPET. Multivariable logistical regression modeling was used to analyze the associations between interventricular dyssynchrony and risk assessment. RESULTS: Sixty-six PcPH patients were consecutively recruited (19 male and 47 female, average 35 years old). WHO functional class, N-terminal pro-brain natriuretic peptide (BNP) and body mass index were included as independent predictors in the first multivariate regression analysis of clinical data without echocardiographic parameters (Model-1, r2=0.423, P<0.001). We subsequently added conventional echocardiographic parameters and 2D-STE parameters to the clinical data, RV fractional area change (Model-2, r2=0.417, P<0.001), RV global longitudinal strain (Model-3, r2=0.454, P=0.001), RV intraventricular dyssynchrony (Model-4: r2=0.474, P<0.001) and interventricular dyssynchrony (Model-5, r2=0.483, P<0.001) were identified as independent predictors of PVO2. Interventricular dyssynchrony, calculated as the SD of the time intervals of nine segments, was independently associated with risk assessment (odd ratio 1.027, 95% CI: 1.003-1.052, P=0.03). The area under the receiver-operating characteristic curve (AUC) was 0.73 (P<0.001). CONCLUSIONS: Interventricular dyssynchrony detected by 2D-STE contributed to a better evaluation of exercise capacity and disease severity in PcPH patients.

The value of three-dimensional echocardiography in risk stratification in pulmonary arterial hypertension: a cross-sectional study.

To explore the value of right ventricular (RV) parameters detected by three-dimensional echocardiography (3DE) in risk stratification in pulmonary arterial hypertension (PAH) patients. We prospectively recruited 130 pulmonary hypertension patients from National Center for Cardiovascular Diseases, Fuwai Hospital. Each participant was performed a transthoracic echocardiography and 3DE parameters were measured using an off-line software (4D RV Function 2.0, TomTec). Patients were classified into low, intermediate-high risk group based on 2015 ESC Guidelines. A total of 91 PAH patients (34 ± 12 years old, 25 males) were enrolled, among which, 42 were classified into low risk group, while 49 were intermediate-high risk group. Compared with low-risk patients, those with intermediate-high risk had significantly larger 3DE-RV volumes, worse ejection fraction (EF) and tricuspid annular plane systolic excursion, and decreased longitudinal strain (LS). Receive operating characteristic curves illustrated all the 3DE parameters were able to predict intermediate-high risk stratification, especially 3D-RVEF (area under curve, 0.82, 95% CI 0.73-0.91, P < 0.001). And 3D-RVEF < 26.39% had a 81.6% sensibility and 73.8% specificity to predict intermediate-high risk stratification. Univariate and multivariate Logistic regression analyses identified 3D-RV end-diastolic (OR 1.02, 95% CI 1.01-1.03, P = 0.002) and end-systolic (OR 1.03, 95% CI 1.01-1.04, P < 0.001) volumes, 3D-RVEF (OR 0.82, 95% CI 0.75-0.90, P < 0.001) and LS of free wall (OR 1.17, 95% CI 1.05-1.31, P = 0.005) as independent predictors of intermediate-high risk stratification. In conclusion, RV volumes, EF and free wall strain detected by 3DE were independent predictors of intermediate-high risk stratification in PAH patients, among which, RVEF showed the best predictive capacity.

Two-dimensional speckle tracking echocardiography assessed right ventricular function and exercise capacity in pre-capillary pulmonary hypertension.

Resting two-dimensional speckle tracking echocardiography (2D-STE) identified right ventricular (RV) systolic function were reported to predict exercise capacity in pulmonary hypertension (PH) patients, but little attention had been payed to 2D-STE detected RV diastolic function. Therefore, we aim to elucidate and compare the relations between 2D-STE identified RV diastolic/systolic functions and peak oxygen consumption (PVO2) determined by cardiopulmonary exercise testing (CPET) in pre-capillary PH. 2D-STE was performed in 66 pre-capillary PH patients and 28 healthy controls. Linear correlation and multivariate regression analyses were performed to evaluate and compare the relations between RV 2D-STE parameters and PVO2. Receiver operating characteristic curves were used to compare the predictive value of 2D-STE parameters in predicting the cut-off-PVO2 < 11 ml/min/kg. There were significant differences of all the 2D-STE parameters between PH patients and healthy controls. In patients, RV-peak global longitudinal strain (GLS, rs = - 0.498, P < 0.001), RV- peak systolic strain rate (GSRs, rs = - 0.537, P < 0.001) and RV- peak early diastolic strain rate (GSRe, rs = 0.527, P < 0.001) significantly correlated with PVO2, but no significant correlation was observed between RV- peak late diastolic strain rate (GSRa, rs = 0.208, P = 0.093) and PVO2. The first multivariate regression analysis of clinical data without echocardiographic parameters identified WHO functional class, NT-proBNP and BMI as independent predictors of PVO2 (Model-1, adjusted r2 = 0.421, P < 0.001); Then we added conventional echocardiographic parameters and 2D-STE parameters to the clinical data, identified S,(Model-2,adjusted r2 = 0.502, P < 0.001), RV-GLS (Model-3, adjusted r2 = 0.491, P < 0.001), RV-GSRe (Model-4, adjusted r2 = 0.500, P < 0.001) and RV-GSRs (Model-5, adjusted r2 = 0.519, P < 0.001) as independent predictors of PVO2, respectively. The predictive power was increased, and Model-5 including RV-GSRs showed the highest predictive capability. ROC curves found RV-GSRs expressed the strongest predictive value (AUC = 0.88, P < 0.001), and RV-GSRs > - 0.65/s had a 88.2% sensibility and 82.2% specificity to predict PVO2 < 11 ml/min/kg. 2D-STE assessed RV function improves the prediction of exercise capacity represented by PVO2 in pre-capillary PH.

Comparison of the capability of risk stratification evaluation between two- and three-dimensional speckle-tracking strain in pre-capillary pulmonary hypertension.

To investigate and compare the value of right ventricular longitudinal strain detected by two-dimensional and three-dimensional speckle-tracking echocardiography in risk stratification evaluation in pre-capillary pulmonary hypertension. We consecutively screened 66 patients diagnosed with pre-capillary pulmonary hypertension in our center. According to the risk assessment recommended by 2015 European Society of Cardiology Guidelines, all participants were classified into low- and intermediate-high-risk group. Two-dimensional and three-dimensional strains were measured using off-line softwares (GE EchoPAC version 201 and TomTec, 4D RV Function 2.0). Fifty-seven pre-capillary pulmonary hypertension patients (average 35 years old, 18 males and 39 females) were finally enrolled in our study, 32 (56.1%) were classified in low-risk group, while 25 (43.9%) were in the intermediate-high-risk group. Clinical data associated with disease severity, such as N-terminal pro-brain natriuretic peptide (r = 0.574, P < 0.001), peak oxygen consumption (r = -0.484, P < 0.001), and 6-min walking distance (r = -0.356, P = 0.008) were significantly correlated with two-dimensional right ventricular longitudinal strain; while the correlations with three-dimensional right ventricular longitudinal strain were weaker. Receiver operating characteristic curves for the detection of intermediate-high risk stratification showed two-dimensional right ventricular longitudinal strain had the best predictive capacity (area under curve, 0.82, 95% CI: 0.71-0.93, P < 0.001). Univariate and Multivariate Logistic regression analyses identified two-dimensional right ventricular longitudinal strain as an independent predictor (OR: 1.42, 95% CI: 1.18-1.71, P < 0.001) of intermediate-high risk stratification in this cohort of pre-capillary pulmonary hypertension patients, the predictive capacity retained (OR: 1.45, 95% CI: 1.18-1.78, P < 0.001) after adjusted by age, gender, and body mass index, while three-dimensional speckle-tracking echocardiography parameters were not. In conclusion, when used for the detection of intermediate-high risk stratification in pre-capillary pulmonary hypertension, two-dimensional right ventricular longitudinal strain was better than three-dimensional right ventricular longitudinal strain.

Imbalance between tissue inhibitor of metalloproteinase 1 and matrix metalloproteinase 9 after cardiopulmonary resuscitation.

AIMS: This study aimed to determine whether (a) there was an imbalance between matrix metalloproteinase 9 (MMP-9) and tissue inhibitor of metalloproteinase 1 (TIMP-1) after cardiopulmonary resuscitation (CPR) in a canine model of prolonged ventricular fibrillation (VF); (b) with the duration of VF, the degree of the imbalance would be greater; and (c) there was a relationship between the level of MMP-9 or TIMP-1 and the cardiac function. METHODS AND RESULTS: Ventricular fibrillation was electrically induced in 24 dogs. The animals were randomly divided into 3 groups (sham control, n = 8; 8-minute VF, n = 8; 12-minute VF, n = 8). Echocardiographic measurement and hemodynamic variables were recorded before VF and after return of spontaneous circulation. Tissue inhibitor of metalloproteinase 1 (TIMP-1) and MMP-9 were analyzed by Western blot and immunohistochemistry. Compared with sham controls, dogs under VF and CPR showed significantly decreased level of TIMP-1 (P < .001), and with the duration of VF, the level of TIMP-1 declined (P < .01). The level of MMP-9 did not achieve statistical significance in the 3 groups (P > .05); however, they were higher in VF and longer duration VF groups. The ratios of TIMP-1/MMP-9 were lower in VF groups (P < .05). There was a negative correlation between TIMP-1 and left atrium dimension and left ventricular diastolic dimensions (r = -0.83 and r = -0.96, respectively; P < .01) and a positive correlation between TIMP-1 and left ventricular ejection fraction (r = 0.85; P < .01). CONCLUSIONS: There was an imbalance between TIMP-1 and MMP-9 after CPR. It may partly contribute to the postresuscitation cardiac dysfunction.

Effect of ZP123, a gap junction modifier, on prolonged ventricular fibrillation in swine.

OBJECTIVES: It was the aim of this study to investigate the effect of ZP123 on prolonged ventricular fibrillation (VF) in swine. METHODS: VF was electrically induced in 20 pigs. The animals randomly received either ZP123 or saline control infusion before VF. After 8 min of untreated VF, cardiopulmonary resuscitation and biphasic defibrillation shocks were applied. VF mean frequency (VF(mf)) and mean amplitude (VF(ma)), hemodynamics, outcome of defibrillation and the rate of return of spontaneous circulation (ROSC) were analyzed. RESULTS: Compared with the control group, VF(mf) was higher but VF(ma) lower during the 8 min of VF in the drug group (11.8 ± 2.1 vs. 10.4 ± 2.0 Hz and 0.24 ± 0.10 vs. 0.31 ± 0.16 mV, respectively; p < 0.05). Hemodynamic variables in the 2 groups were comparable (p > 0.05). The defibrillation threshold was lower and the rate of successful defibrillation was higher in the drug group compared with the control group (92.2 ± 26.4 vs. 133.3 ± 28.9 J and 90 vs. 30%, respectively; p < 0.05). The rate of ROSC was not different between the 2 groups (40 vs. 30%; p > 0.05). CONCLUSION: In prolonged VF, ZP123 could decrease the defibrillation threshold and improve the rate of successful defibrillation. However, it could not improve the rate of ROSC - which may be due to its side effect of decreasing VF(ma).

Level Of Natriuretic Peptide Determines Outcome In Atrial Fibrillation.

Background: Natriuretic peptide (NP) is high in atrial fibrillation (AF) and may decrease after cardioversion to sinus rhythm and the levels of atrial NP (ANP) and brain NP (BNP) in different types of AF and whether ANP and BNP have predictive values for relapsed AF have not been determined. Purpose: We aimed to examine the levels of ANP and BNP in AF to determine their roles in different types of AF, including a predictive value in relapsed AF. Methods and Results: ANP and BNP were measured in 100 consecutive patients with AF and without heart dysfunction at baseline and in 20 controls. All patients had higher levels than controls (p<0.01). After cardioversion treatment with antiarrhythmic therapy, 40 patients failed to cardioversion successfully and still showed AF, whereas 60 patients were successful. ANP and BNP levels decreased significantly after cardioversion (163.55±54.27pg/ml vs. 200.20±55.63 pg/ml; 124.15±43.00 pg/ml vs. 161.99±48.04 pg/ml, for ANP and BNP respectively, both p<0.0001). 18 of the 60 successfully cardioverted patients had AF recurred within 24 hours, who were then excluded from 500-day follow-up and the remaining 42 patients were enrolled. During 500-day follow-up period, AF relapsed in 16 patients. Comparing with the 42 patients, the 16 patients showed higher concentrations of ANP (187.72±32.79 pg/ml vs. 138.42±30.65 pg/ml, p<0.0001). Besides, both ANP and BNP were significantly higher in the relapsed patients than those remained SR during follow-up (153.38±29.61pg/ml vs. 129.21±27.98pg/ml for ANP, p=0.01 and 147.41±25.95pg/ml vs. 121.87±20.53pg/ml for BNP, p=0.001). The area under the receiver-operating characteristic curve was 0.799 for BNP and 0.706 for ANP in predicting a relapse of AF. Using the BNP optimized cut-off level of 138 pg/ml, relapsed AF can be predicted with relatively acceptable accuracy. CONCLUSIONS: ANP and BNP decrease significantly after cardioversion in patients with AF, and both can be useful predictors of relapsed AF.