One-minute sit-to-stand test in patients with pulmonary arterial hypertension associated with congenital heart disease: A single-center prospective study.

BACKGROUND: Patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) have reduced exercise capacity. Recently, the 1-minute sit-to-stand test (1MSTST), which measures the number of repetitions from sitting to standing position in 1 minute, has been proposed as an alternative test to the 6-minute walking test (6MWT). The aim of our study was to assess the safety and results of the 1MSTST in comparison to the 6MWT in patients with PAH-CHD. METHODS: Consecutive adult patients with PAH-CHD underwent the 6MWT and the 1MSTST on the same day. The 6-minute walking distance in meters and the number of repetitions on the 1MSTST were measured. Heart rate, peripheral oxygen saturations, Borg dyspnea score, and lower limb fatigue were recorded before and immediately after testing. Correlations between both tests and clinical, laboratory, and imaging parameters were statistically analyzed. RESULTS: The study included 40 patients (50% female, mean age 43 ± 15 years), of whom 29 (72%) had Eisenmenger syndrome and 14 (35%) had Down syndrome. The number of 1MSTST repetitions correlated significantly with 6MWT distance (r = 0.807, p = 0.000). There were no adverse events, and the 1MSTST results correlated with the WHO functional class. Heart rate increase and oxygen desaturation after both tests correlated significantly, but less desaturation was observed after 1MSTST. CONCLUSIONS: Our study showed that the 1MSTST is a safe and easily applicable test in adult patients with PAH-CHD, including patients with Down syndrome. The results of the 1MSTST correlate significantly with the 6MWT, providing an alternative tool for exercise capacity assessment in patients with PAH-CHD.

Effect of Coronary Sinus Reducer Implantation on Aerobic Exercise Capacity in Refractory Angina Patients-A CROSSROAD Study.

Coronary sinus reducer (CSR) implantation is a new treatment option for patients with refractory angina pectoris. However, there is no evidence from a randomized trial that would show an improvement in exercise capacity after this treatment. The aim of this study was to evaluate the influence of CSR treatment on maximal oxygen consumption and compare it to a sham procedure. Twenty-five patients with refractory angina pectoris (Canadian Cardiovascular Society (CCS) class II-IV) were randomized to a CSR implantation (n = 13) or a sham procedure (n = 12). At baseline and after 6 months of follow-up, the patients underwent symptom-limited cardiopulmonary exercise testing with an adjusted ramp protocol and assessment of angina pectoris using the CCS scale and Seattle angina pectoris questionnaire (SAQ). In the CSR group, maximal oxygen consumption increased from 15.56 ± 4.05 to 18.4 ± 5.2 mL/kg/min (p = 0.03) but did not change in the sham group (p = 0.53); p for intergroup comparison was 0.03. In contrast, there was no difference in the improvement of the CCS class or SAQ domains. To conclude, in patients with refractory angina and optimized medical therapy, CSR implantation may improve oxygen consumption beyond that of optimal medical therapy.

The association between myocardial ischemia and myocardial dysfunction in adult patients with systemic right ventricle - A single centre multimodality study.

BACKGROUND: The exact interaction of factors leading to myocardial dysfunction and fibrosis of the systemic right ventricle (SRV) is not completely understood. Myocardial ischemia and injury associated with a supply-demand mismatch of the pressure overloaded SRV are thought to play an important role, however studies confirming this are lacking. METHODS: Adult SRV patients were included in this single centre cohort study. All patients underwent a comprehensive diagnostic and imaging workup. A two-day stress-rest SPECT was performed to assess myocardial perfusion. SRV ischemia was defined as decreased segmental tracer uptake during exercise with significant improvement at rest. Contrast enhanced cardiac magnetic resonance imaging (CMR) was also performed in a subgroup of patients without contraindication, to assess focal myocardial fibrosis. Differences between patients with and without SRV ischemia were assessed. RESULTS: Twenty-three SRV patients (15 with transposition of the great arteries after atrial switch procedure and 8 with congenitally corrected transposition of the great arteries; 5 (22%) females; mean age 38 ± 11 years) were included. Seven (30%) patients had SRV ischemia on SPECT. Late gadolinium enhancement on CMR was more common in patients with SRV ischemia (p = 0.002). However, there was no association between SRV ischemia and different echocardiographic or CMR parameters of SRV systolic function, laboratory markers (high-sensitivity troponin I and NT-proBNP) and exercise capacity. CONCLUSIONS: Our multimodality study showed that SRV ischemia in adult SRV patients was associated with more focal myocardial fibrosis, but not with functional or imaging markers of SRV function.

Cardiac myxoma: single tertiary centre experience.

BACKGROUND: Although cardiac myxoma (CM) are rare and benign, they can cause life-threatening complications, such as hemodynamic disturbances or embolization. Surgical excision of the tumour is the treatment of choice. The aim of the study was to evaluate the epidemiological characteristics, clinical presentation, imaging findings, and outcomes of surgical treatment of patients with CM treated in the largest tertiary care centre in Slovenia. PATIENTS AND METHODS: We retrospectively analysed the medical records of all patients referred to our institution between January 2005 and December 2020 and identified 39 consecutive adult patients with pathologically confirmed CM. RESULTS: The average annual incidence of CM in the study was 3 per 2 million population per year. Patients were more often female (n = 25, 64%). The mean age at diagnosis was 63.1 ± 13.6 years. Dyspnoea was the most common presenting symptom (31%). CM was an incidental finding in 11 patients (28%). Seven patients presented with thromboembolic event (18%). Transthoracic echocardiography (TTE) was performed in all patients, however additional imaging was required in 22 patients (56%). All patients in our series were successfully treated surgically without in-hospital mortality. During the follow-up period (6 months to 16 years) three patients (8%) died, and all deaths were unrelated to CM. There was no recurrence of CM during the follow-up. CONCLUSIONS: Our single-centre study confirms that CM is rare cardiac tumour with diverse clinical presentation. Our data shows data that CM might be more prevalent than considered before. Surgical resection of the tumour is safe with excellent short- and long-term outcomes.

Efficacy of coronary sinus reducer implantation in patients with chronic total occlusion of the right coronary artery.

BACKGROUND: Clinical efficacy of coronary sinus reducer (CSR) in refractory angina (RA) patients with ischemia due to the chronic total occlusion (CTO) of the right coronary artery (RCA) remains unknown. AIMS: To evaluate the efficacy of CSR implantation in RA patients with CTO RCA and compare them to CSR recipients with left coronary artery (LCA) ischemia. METHODS: Consecutive patients with CTO RCA from 2 centers were prospectively included and compared to patients with LCA ischemia. All patients underwent evaluation of angina severity and quality of life (QoL) at baseline and after 12 months. In a subgroup of CTO RCA patients, stress cardiac magnetic resonance (CMR) imaging was also performed. RESULTS: Twenty-two patients with CTO RCA and predominant inferior and/or inferoseptal wall ischemia (the CTO RCA group) were compared to 24 patients with predominant anterior, lateral, and/or anteroseptal wall ischemia (the LCA group). While the Canadian Cardiovascular Society (CCS) anginascore mean (SD) improved in the CTO RCA group from 2.73 (0.46) to 1.82 (0.73) (P <0.001) and in the LCA group from 2.67 (0.57) to 1.92 (0.72) (P <0.001), there was no intergroup difference (P = 0.350). Significant improvement in all domains of the Seattle Angina Questionnaire was observed. Stress CMR did not show a significant reduction of ischemic inferior and/or inferoseptal segments, however, improvements in the transmurality index (P = 0.03) and the myocardial perfusion reserve index in segments with inducible ischemia (P = 0.03) were observed in the CTO RCA group. CONCLUSIONS: In CTO RCA patients, CSR implantation alleviated angina symptoms and improved QoL. The extent of improvement was comparable to that observed in patients with LCA ischemia.

Resynchronization therapy with His bundle pacing in a patient after coronary sinus reducer implantation.

Symptomatic heart failure patients with ischemic heart disease may require both coronary sinus reducer (CSR) implantation due to refractory angina pectoris and cardiac resynchronization therapy (CRT). Optimal approach to CRT in these patients is unknown as CSR implantation in the distal coronary sinus could deter left ventricular lead placement and thus preclude conventional CRT with biventricular pacing. We present a 70-year-old patient with ischemic cardiomyopathy and wide QRS complex after CSR implantation in whom we achieved successful cardiac resynchronization with His bundle pacing (HBP). HBP led to acute improvement in hemodynamic parameters and exercise capacity that persisted at follow-up. This case represents the first description of successful CRT with HBP in a patient after CSR implantation. HBP could present a feasible and safe resynchronization approach in these patients. .

Coronary Artery Embolism From a Blood Cyst of the Mitral Valve.

Blood cysts are benign, congenital tumours of the heart endothelium, found most commonly on heart valves. Adult cases are rare, since most spontaneously regress with age. However, reports of symptomatic cases with embolic phenomena and valve dysfunction have been described. We present a case of a previously healthy 44-year-old woman with no cardiovascular risk factors who developed acute myocardial infarction caused by coronary artery embolism from a blood cyst of the anterior mitral valve leaflet.