Impact of diabetes mellitus on disease severity and patient survival in idiopathic pulmonary arterial hypertension: data from the Polish multicentre registry (BNP-PL).

BACKGROUND: Recent studies revealed that alterations in glucose and lipid metabolism in idiopathic pulmonary arterial hypertension (IPAH) are associated with disease severity and poor survival. However, data regarding the impact of diabetes mellitus (DM) on the prognosis of patients with IPAH remain scarce. The aim of our study was to determine that impact using data from a national multicentre prospective pulmonary hypertension registry. METHODS: We analysed data of adult patients with IPAH from the Database of Pulmonary Hypertension in the Polish population (BNP­PL) between March 1, 2018 and August 31, 2020. Upon admission, clinical, echocardiographic, and haemodynamic data were collected at 21 Polish IPAH reference centres. The all-cause mortality was assessed during a 30-month follow-up period. To adjust for differences in age, body mass index (BMI), and comorbidities between patients with and without DM, a 2-group propensity score matching was performed using a 1:1 pairing algorithm. RESULTS: A total of 532 patients with IPAH were included in the study and 25.6% were diagnosed with DM. Further matched analysis was performed in 136 patients with DM and 136 without DM. DM was associated with older age, higher BMI, more advanced exertional dyspnea, increased levels of N-terminal pro-brain natriuretic peptide, larger right atrial area, increased mean right atrial pressure, mean pulmonary artery pressure, pulmonary vascular resistance, and all-cause mortality compared with no DM. CONCLUSIONS: Patients with IPAH and DM present with more advanced pulmonary vascular disease and worse survival than counterparts without DM independently of age, BMI, and cardiovascular comorbidities.

Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL).

Current knowledge of pulmonary arterial hypertension (PAH) epidemiology is based mainly on data from Western populations, and therefore we aimed to characterize a large group of Caucasian PAH adults of Central-Eastern European origin. We analyzed data of incident and prevalent PAH adults enrolled in a prospective national registry involving all Polish PAH centers. The estimated prevalence and annual incidence of PAH were 30.8/mln adults and 5.2/mln adults, respectively and they were the highest in females ≥65 years old. The most frequent type of PAH was idiopathic (n = 444; 46%) followed by PAH associated with congenital heart diseases (CHD-PAH, n = 356; 36.7%), and PAH associated with connective tissue disease (CTD-PAH, n = 132; 13.6%). At enrollment, most incident cases (71.9%) were at intermediate mortality risk and the prevalent cases had most of their risk factors in the intermediate or high risk range. The use of triple combination therapy was rare (4.7%). A high prevalence of PAH among older population confirms the changing demographics of PAH found in the Western countries. In contrast, we found: a female predominance across all age groups, a high proportion of patients with CHD-PAH as compared to patients with CTD-PAH and a low use of triple combination therapy.

An assessment of regression of left ventricular hypertrophy following alcohol ablation of the interventricular septum in patients with hypertrophic cardiomyopathy with left ventricular outflow tract obstruction.

BACKGROUND: Hypertrophic obstructive cardiomyopathy (HOCM) is characterised by asymmetric myocardial hypertrophy, which is most pronounced in the interventricular septum (IVS) and is responsible for the dynamic obstruction of the left ventricular outflow tract (LVOT). Successful alcohol septal ablation (ASA) of the IVS allows to reduce the thickness of the parabasal part of the IVS myocardium and, in most cases, to permanently reduce the gradient in the LVOT. AIM: To assess, using cardiac magnetic resonance imaging (MRI) and transthoracic echocardiography (TTE), the impact of gradient reduction in the LVOT on the type and severity of left ventricular (LV) remodelling. METHODS: The study included 30 patients (aged 56.9 ± 11.9 years) with HOCM and the mean peak gradient (PG) in the LVOT of 123 ± 33 mm Hg who underwent ASA. MRI measurements were performed before and at 6 months after ASA and TTE measurements were performed before, at 3 months and at 6 months after ASA. RESULTS: PG in the LVOT decreased to an average of 52 ± 37 mm Hg (p 〈 0.0001) at 3 months after ASA and to 37 ± 28 mm Hg (p 〈 0.0001) at 6 months after ASA. TTE revealed a decrease in IVS thickness outside the scar following ASA from 23.6 ± 3.5 mm to 19.3 ± 4.0 mm (p 〈 0.0001) and 19.4 ± 0.4 mm (p 〈 0.0001) at 3 and 6 months, respectively. There was also a decrease in lateral wall (PW) thickness from 15.9 ± 3.2 mm to 14.9 ± 2.9 mm (p = 0.046) and 14.16 ± 2.00 (p = 0.0065) at 3 and 6 months, respectively. MRI revealed a decrease in IVS thickness from 23.7 ± 2.8 mm to 18.04 ± 4.00 mm (p = 0.0001) at 6 months following ASA. We observed a regression of the PW hypertrophy from 13.2 ± 3.35 mm to 12.18 ± 2.4 mm (p = 0.0225). There was a decrease in IVS mass from 108.9 ± 20 g to 91.5 ± 29 g (p = 0.0006). There was a trend towards a decreased LV mass and LV mass excluding IVS mass at 6 months. CONCLUSIONS: A significant decrease in PG in the LVOT is associated with a decrease in LV mass and with regression of LV hypertrophy outside the scar after ASA.

Long term exercise capacity in patients with hypertrophic cardiomyopathy treated with percutaneous transluminal septal myocardial ablation.

BACKGROUND: In hypertrophic obstructive cardiomyopathy, percutaneous transluminal septal myocardial ablation (PTSMA) improves functional capacity in the short term. However, long term functional capacity is unknown. AIM: To assess the long term exercise capacity of patients with hypertrophic obstructive cardiomyopathy undergoing PTSMA. METHODS: Twenty three patients (56.5% male, mean age 44.5+/-13.6 years) who underwent PTSMA were included. All patients had also undergone a symptom limited cardiopulmonary exercise treadmill test before the procedure, then after 3 months (early follow-up) and after a mean 7.2+/-1.0 years (long term follow-up). RESULTS: Before PTSMA, mean maximal pressure gradient in the left outflow tract (LVOTGmax) was 82+/-29 mmHg, 17 patients had NYHA functional class> or = III and peak oxygen uptake (pVO2) was 18+/-4 ml/kg/min. PTSMA led to a reduction in mean LVOTGmax (to 29+/-19 mmHg, p<.0001), improvement of heart failure symptoms (NYHA> or =III in 1 patient, p<.0001) and an increase of pVO2 (to 22+/-6 ml/kg/min, p=.0002) at short term. LVOTGmax, functional class and pVO2 did not change significantly during long term follow-up compared to early follow-up. However, there was a continuous improvement in percentage predicted pVO2 over time. CONCLUSIONS: In patients with hypertrophic obstructive cardiomyopathy and symptoms of heart failure, PTSMA leads to stable long term improvement of objectively measured exercise capacity.

Reduction of the QT interval dispersion after aortic valve replacement reflects changes in electrical function rather than structural remodeling.

BACKGROUND AND AIM OF THE STUDY: Factors related to changes of QT dispersion (QTd) after aortic valve replacement (AVR) in patients with aortic stenosis were analyzed. METHODS: The prospective group comprised 121 consecutive patients (45 women, 76 men; mean age 58 +/- 11 years; range: 24-77 years) with significant aortic valve stenosis. Data (clinical, echocardiographic and electrocardiographic) were collected before and at least 16 months after AVR. QTd was measured in the standard ECG. RESULTS: Before AVR, the mean QTd was 60 +/- 24 ms (QT(max) 424 +/- 40 ms). QTd was > 50 ms in 68% of patients, and > 70 ms in 30%. During postoperative follow up the mean QTd was 54 +/- 19 ms (QT(max) 368 +/- 36 ms) for all patients, and was > 50 ms in 58% of cases and > 70 ms in 13%. Postoperatively, QTd was decreased to < 70 ms in 27% of patients with a normalized left ventricular mass index (LVMI), and in 27% of those without any clinically significant reduction in left ventricular (LV) hypertrophy. In the multivariate analysis, QTd reduction was weakly related to the reduction in LV wall thickness (p = 0.09) and LVMI (p = 0.05). The reduction in QTd was more related to changes in T-wave amplitude in lead V5 (p = 0.004). CONCLUSION: Following AVR for aortic stenosis, a decrease in QTd was observed, notably among patients with QTd > 70 ms. This reduction was only weakly related to the degree of reduction in cardiac hypertrophy, but a more important relationship was observed with changes in T-wave amplitude. These findings suggest that a reduction in QTd after AVR is reflective of changes in electrical function rather than structural remodeling.

QT interval dispersion analysis in patients with aortic valve stenosis: a prospective study.

BACKGROUND AND AIM OF THE STUDY: QT interval dispersion (QTd) was analyzed in patients with aortic valve stenosis, and the relationship investigated between QTd, age, gender, hypertension, presence of significant coronary artery stenosis and left ventricular (LV) hypertrophy assessed by echocardiography. Results were also compared between survivors and patients who had cardiac arrest or died before, during or soon after cardiac surgery. METHODS: The group comprised 535 consecutive patients (217 women, 318 men; mean age 59 +/- 11 years; range: 20-81 years) with significant aortic valve stenosis before valve replacement. The control group comprised 35 healthy subjects (12 women, 23 men; mean age 51 +/- 11 years; range: 28-74 years). RESULTS: Mean QTd was 29 +/- 10 ms in controls and 59 +/- 24 ms in patients (p < 0.001). Gender had no impact on QTd. QTd was increased in men with significant coronary artery stenosis, and independently related strongly with ECG parameters (QTmax, heart rate) and weakly with age and degree of LV hypertrophy. In patients with cardiac arrest or those who died (n = 14), QTd was increased compared to that in survivors (71 +/- 22 versus 59 +/- 24 ms; p = 0.05), and QTd >70 ms was observed more frequently (p = 0.02; odds ratio 3.4, 1.16-10.0). CONCLUSION: QTd is abnormally increased in two-thirds of patients with aortic valve stenosis, and is increased in men with concomitant coronary artery disease. QTd >70 ms significantly increased the risk of cardiac arrest or death perioperatively. QTd was only weakly related with age and degree of LV hypertrophy, but QTmax and heart rate had a greater impact. QT dispersion analysis has limited clinical value in patients with aortic stenosis.