Background We performed a systematic review of cor triatriatum sinistrum (CTS) diagnosed in adults. The aim of this review was to describe the clinical presentation, natural history and management of this congenital heart disease. Methods A PubMed literature search for ‘cor triatriatum sinistrum’ published since 2005 was performed. Included patients were divided into those with and without obstructive membrane physiology. The clinical course differences were compared. Results A total of 171 published cases were included. The median age at diagnosis was 43 years (IQR, 30–60). Obstructive membrane physiology was observed in 70 (41%), and this patient group was younger at presentation (median age 39 (IQR, 28–52) vs 50 years (IQR, 32–64), p=0.003). Patients with obstructive membrane more frequently had associated cardiac defects (58.6% vs 42.4%, p=0.039). Overall, the most frequent clinical symptom was atrial fibrillation, as this was present in 56 (32.8%) of all patients. CTS-related symptoms were more frequent in patients with obstructive membrane: congestive heart failure (44.3% vs 15.2%, p<0.001), pulmonary hypertension (27.1% vs 6.1%, p<0.001), haemorrhage (8.6% vs 0%, p=0.004) and infections manifestation (8.6% vs 0%, p=0.004). A total of 71 (41.5%) patients with CTS required interventional treatment, mainly within patients with the obstructive membrane (86.8% vs 12.6%, p<0.001). Conclusion The natural history of CTS most often manifests with symptoms of congestive heart failure. Patients with obstructive membrane most often have associated cardiac defects and higher risk for infections and haemorrhage. The interventional treatment of CTS remains the first choice for obstructive membrane.

Background and Objectives: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients’ well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging. The study aimed to share our experience with PAH-CHD pregnancies and discuss the risk assessment and current management of these patients with the combination of two rare diseases. Materials and Methods: The retrospective search of pulmonary hypertension and adult CHD registries in our hospital was performed, selecting all patients with CHD and PAH who conceived pregnancy from 2013 to 2021. Baseline demographic, clinical, and functional characteristics and clinical outcomes were collected. Results: Thirteen pregnancies in eight patients with PAH-CHD resulted in seven live births, three miscarriages, and three terminations. Five women were diagnosed with Eisenmenger syndrome (ES) and three with residual PAH after CHD repair. Before pregnancy, half of them were in WHO functional class III. Seven (87.5%) patients received targeted PAH treatment with sildenafil during pregnancy. In addition, the two most severe cases were administered with iloprost during peripartum. Three ES patients delivered preterm by Caesarean section under general anaesthesia. No neonatal mortality was reported. Maternal complications were observed in half of our cases. One patient died 12 days after the delivery in another hospital due to deterioration of heart failure. Conclusions: On the basis of our clinical experience, we conclude that pregnancy and delivery carry a high risk for maternal complications and should be avoided in women with PAH-CHD. The individualised approach of multidisciplinary care and appropriate monitoring are mandatory in reducing the risk of adverse outcomes.

Background and Objectives: After successful surgical repair of a congenital shunt lesion, pulmonary hypertension (PH) often disappears. However, PH can persist long-term after the closure. This study aimed to assess the prevalence of PH long-term after surgical repair of congenital heart disease (CHD), and to evaluate the outcomes and preoperative factors related to residual PH. Materials and Methods: In this retrospective cohort study, we reviewed patients who underwent right heart catheterisation in Vilnius University Hospital Santaros Klinikos during the period of 1985–2007. Among 4118 right heart catheterisations performed, 160 patients underwent congenital systemic-to-pulmonary shunt repair at a young age (<18 years) and had pre-operative PH. Half of the patients were foreigners whose follow-up data were unavailable. Eventually, 88 patients with available follow-up data were included in this study. Results: The median age at diagnosis of CHD with PH was 0.8 (0.6–3.0) and 1.1 (0.6–3.9) years at surgery (50% females). Residual PH was assessed 9.5 years after surgery and observed in 30.7% (n = 27) of the patients. It was associated with having more than one shunt (44.4% (n = 12), p = 0.016) and higher median pulmonary vascular resistance (3.4 (2.5–6.5) vs. 2.2 (1.0–3.7), p = 0.035) at baseline. After a median follow-up of 21 (15–24) years, 9.1% of the patients were deceased. Kaplan–Meier survival analysis revealed significantly higher mortality in the residual PH group (p = 0.035). Conclusions: Residual PH affects a significant proportion of patients after surgical repair of a shunt lesion and is associated with worse long-term outcome.

(2017). Challenging diagnosis of prosthetic infective endocarditis and para-aortic conduit abscess – the role of multimodality approach. Acta Cardiologica: Vol. 72, No. 6, pp. 669-670.

Background and Objectives. COVID-19 infection has a significant burden on global morbidity and mortality, especially in elderly people and in patients with chronic respiratory and cardiovascular diseases, such as pulmonary hypertension (PH). We aimed to evaluate the impact of COVID-19 infection on patients diagnosed with severe chronic PH. Materials and Methods. A single-center prospective cohort study was performed. Patients were enrolled from 1 November 2020 to 31 December 2022. Follow-up was until 31 December 2023. Data were collected on PH diagnosis, clinical presentation, outcomes, brain natriuretic peptide (BNP) levels, pulmonary function test with lung diffusion capacity for carbon monoxide (DLCO), and computed tomography pulmonary angiography (CTPA) analysis. Results. During the 26 months of our study, 51 PH patients were diagnosed with COVID-19 infection. The majority, 44 (86.3%) of all COVID-19 infected patients, were treated on an outpatient basis, and 7 (13.7%) required hospitalization. During the follow-up period, 8 (15.7%) patients died: 4 (7.8%) due to complications of COVID-19 infection, and the other 4 (7.8%) died in the later stages of the follow-up period after recovery from acute COVID-19 infection. Therefore, the in-hospital mortality in our study was 43% (n = 3). As mentioned above, the overall mortality was 7.8% (n = 4). Higher BNP levels in the third month after COVID-19 were associated with higher mortality rates (p = 0.028). Lung function, including DLCO, did not significantly worsen with COVID-19. In our study, 24 patients (47.1%) were referred for a follow-up CTPA scan and one of them developed typical fibrotic lung changes after COVID-19. Conclusions. The incidence of COVID-19 infection in patients diagnosed with PH was 34%. In our patients with severe chronic PH, the overall mortality rate due to COVID-19 infection was low. Pulmonary fibrosis was a rare complication in our cohort. COVID-19 infection in severe PH may increase the risk of worsening chronic heart failure.