Efficacy of peritoneal dialysis in patients with refractory congestive heart failure: a systematic review and meta-analysis.

Refractory congestive heart failure (RCHF) is a common complication in the natural history of advanced heart failure. Peritoneal dialysis (PD) is a possible alternative in those patients, but studies are scarce, and mostly with small samples. We conducted this meta-analysis to evaluate the effects of PD in patients with RCHF. Articles published before July 2020 in the following databases: PubMed, Web of Science, and CENTRAL. Mean differences (MD) and 95% confidence intervals (CIs) were computed to generate a pooled effect size with a random effects model. We also assessed heterogeneity, risk of bias, publication bias, and quality of evidence. Twenty observational studies (n = 769) were included, with a "before and after intervention" design. PD was associated with a significant reduction in NYHA functional class (MD -1.37, 95% CI -0.78 to -1.96) and length of hospitalisation (MD -34.8, 95% CI -20.6 to -48.9 days/patient/year), a small but significant increase in left ventricular ejection fraction (MD 4.3, 95%CI 1.9 to 6.8%) and a non-significant change in glomerular filtration rate (MD -3.0, 95% CI -6.0 to 0 mL/min/1.73m2). Heterogeneity among studies was significant and overall risk of bias was rated from moderate to critical. No significant publication bias was found, and the overall quality of evidence was very low for all outcomes. PD in patients with RCHF improved functional class, length of hospitalisation, and ventricular functional, and had no impact in renal function. Further randomised clinical trials are warranted to confirm our results that showed some limitations.

Outcomes in Pregnant Women with Valvular Heart Disease from Portuguese-Speaking African Countries Treated in Portugal through an International Agreement of Health Cooperation.

Aims: We performed a clinical audit of maternal and fetal outcomes in pregnant women with valvular heart disease (VHD) from Portuguese-speaking African countries who were transferred for their care, during a twenty-year period, through a memorandum of agreement of international cooperation. Methods and results: A retrospective analysis of 81 pregnancies in 45 patients with VHD (median age 24, interquartile range 22-29 years) from 2000 to 2020 was performed. The main outcome measures were maternal cardiovascular and fetal outcomes. History of rheumatic heart disease was present in 60 (74.1%) pregnancies. Most were in New York Heart Association (NYHA) functional class I or II; at the first evaluation, 35 (43.2%) were on cardiac medication and 49 (60.5%) were anticoagulated. Forty-eight pregnancies had at least one valvular prosthesis, including 38 mechanical heart valves. During pregnancy, deterioration in NYHA functional class occurred in 35 (42.0%), and eight (9.9%) patients required initiation or intensified cardiac medication. Mechanical valve thrombosis complicated four (4.9%) pregnancies, all cases on heparin, and resulted in one maternal death. Haemorrhagic complications happened in 7 (8.6%) anticoagulated patients, in the immediate postpartum or puerperal period. The 81 pregnancies resulted in 56 (69.1%) live births, while miscarriage and fetal malformations occurred in 19 (23.5%) and 12 (14.8%) pregnancies, respectively. In multivariate analysis, vitamin K antagonist therapy was the only independent predictor of an unsuccessful pregnancy (p = 0.048). Conclusion: In a high-income country, successful pregnancy was possible with low rate of maternal events in women with VHD transferred from five low-middle income countries in Africa. The use of anticoagulation with a vitamin K antagonist was associated with an unsuccessful pregnancy.

Malignant Primary and Metastatic Cardiac Tumors: A Single-Center 27-Year Case Review.

BACKGROUND: Malignant primary cardiac tumors are exceedingly rare, and despite surgical exeresis or chemotherapy, their prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. This study aimed to review patients diagnosed with malignant primary and secondary cardiac tumors in a tertiary center between 1995 and 2022. METHODS: Clinical data, echocardiographic, computed tomography, and magnetic resonance assessments of tumor location and morphology, histology, treatment, and survival were retrospectively analyzed. RESULTS: Sixty malignant cardiac tumors were diagnosed: 17 primary (A) and 43 metastatic (B) tumors. A: the most common types were angiosarcoma (41%), undifferentiated sarcoma (23%), and fibrosarcoma (18%). Patients with primary tumors were younger than patients with metastatic tumors (41 ± 13 years vs. 57 ± 18 years, p = 0.001), with no significant gender difference. The most frequent presentations were heart failure (59%) and arrhythmia (23%). The most prevalent tumor location was the right heart chambers (71%), mostly in the right atrium (35%). 47% were submitted to tumor resection, and 29% received chemotherapy. The mortality rate was 82% with a median survival of 6.0 (interquartile range: 1.0-11.8) months after diagnosis (minimum of 12 days and maximum of 19 years). One patient with fibrosarcoma underwent heart transplantation and was still alive and well after 19 years. B: regarding metastatic cardiac invasion, the most common primary tumor sites were lung carcinomas (38%), thymomas (17%), and lymphomas (14%). Presentation with pericardial effusion was common (33%). The mortality rate was 72%, with a median survival of 3.6 (1.0-13.4) months (minimum of 7 days, maximum of 5 years). CONCLUSION: Diagnosis of metastatic cardiac tumors was more common than that of malignant primary tumors, both with a dismal prognosis. When radical exeresis is not possible, heart transplantation can be an option with a favorable outcome in carefully selected patients with sarcomas.

Infective endocarditis in a cohort of adult CHD patients.

BACKGROUND: CHD increases the risk of infective endocarditis due to the substrate of prosthetic materials and residual lesions. However, lesion-specific and mortality risks data are lacking. We sought to analyse clinical course and mortality of infective endocarditis in a cohort of adult CHD. METHODS: Retrospective analysis of all cases of proven and probable infective endocarditis (Duke's criteria) followed in our adult CHD clinic between 1970 and August, 2021. Epidemiological, clinical and imaging data were analysed. Predictors of surgical treatment and mortality were assessed using regression analysis. RESULTS: During a mean follow-up of 15.8 ± 10.9 years, 96 patients had 105 infective endocarditis episodes, half with previous cardiac surgery (corrective or palliative). The most frequent diagnoses were: ventricular septal defect, bicuspid aortic valve, Tetralogy of Fallot and pulmonary atresia. The site of infection was identified by echocardiography in 82 episodes (91%), most frequently in aortic (n = 27), tricuspid (n = 15), and mitral (n = 13) valves. Blood cultures were positive in 79% of cases, being streptococci (n = 29) and staphylococci (n = 23) the predominant pathogens. Surgery was necessary in 40% and the in-hospital mortality was 10.5%, associated with heart failure (p < 0.001; OR 13.5) and a non-surgical approach (p = 0.003; OR 5.06). CONCLUSIONS: In an adult CHD cohort, infective endocarditis was more frequent in patients with ventricular septal defect and bicuspid aortic valves, which contradicts the current guidelines that excludes them from prophylaxis. Surgical treatment is often required and mortality remains substantial. Prevention of this serious complication should be one of the major tasks in the care of adults with CHD.

Unrepaired tetralogy of Fallot in the adult: an unexpected diagnosis.

A 40-year-old female is admitted for paroxysmal episodes of exertional dyspnoea, with associated cyanosis, improving with squatting, and a holosystolic murmur radiating to the interscapular area. Echocardiography showed a subaortic ventricular septal defect with left-to-right shunt and overriding aorta. The characteristic murmur prompted us to seek right ventricular outflow tract obstruction. Magnetic resonance was performed, confirming Tetralogy of Fallot, and corrective surgery was performed.

Non-VKA Oral Anticoagulants in Adult Congenital Heart Disease: a Single-Center Study.

PURPOSE: Non-VKA oral anticoagulants (NOACs) prescription is increasing in adults with congenital heart disease (ACHD). However, data on efficacy and safety in ACHD is unclear, particularly in severe CHD. The study aimed to review the safety and efficacy of NOACs in ACHD. METHODS: Retrospective evaluation of ACHD patients started on NOACs from 2014 to 2020, with the primary endpoints of bleeding or thromboembolic events (TE). CHA2DS2-VASc and HAS-BLED scores were calculated, mortality was assessed, and risk factors for bleeding were identified. RESULTS: A total of 93 patients were included, the mean age was 52 ± 15 years, 58% were female, 55.9% had moderate CHD, and 23.7% had severe CHD (3.2% Fontan). Most (66%) had a CHA2DS2-VASc score ≥ 2 and 82% HAS-BLED ≤ 2. In a median follow-up of 41 (IQR 21) months (400.4 patient-years), there were TE in two patients. The annual risk for TE was 0.49%/patient/year. The cardiovascular mortality was 2% and all-cause mortality 5%; there were no fatal TE or bleeding events. Minor (n = 6, 6.5%) and major (n = 3, 3.2%) bleeding events were observed, a median of 12 (IQR 15) months after starting NOAC therapy. The annual risk for bleeding was 2.2%/patient/year. Renal disease (HR 14.6 [95% CI 1.23-73.6], p = 0.033) and the HAS-BLED score were predictors of major (adjusted HR 6.97 [95% CI 1.69-28.78], p = 0.007) and minor (adjusted HR 3.80 [95% CI 1.48-9.78], p = 0.006) bleeding complications. CONCLUSION: In this real-life cohort of selected ACHD, the use of NOACs was safe and effective, with a low incidence of bleeding events.

Cancer patients with acute coronary syndrome have non-superior bleeding risk compared to patients with similar characteristics - a propensity score analysis from the ProACS registry.

INTRODUCTION: The management of acute coronary syndrome (ACS) in malignancy is challenging due to higher bleeding risk. METHODS: We analyzed patients with cancer (active or in the previous five years) prospectively included in the ProACS registry between 2010 and 2019. Our aim was to assess safety (major bleeding, primary endpoint) and secondary efficacy endpoints (in-hospital mortality and combined in-hospital mortality, reinfarction and ischemic stroke) of ACS treatment. Propensity score matching analysis (1:1) was further performed to better understand predictors of outcomes. RESULTS: We found 934 (5%) cancer patients out of a total of 18 845 patients with ACS. Cancer patients had more events: major bleeding (2.9% vs. 1.5%), in-hospital mortality (5.8% vs. 3.4%) and the combined endpoint (7.4% vs. 4.9%). The primary endpoint was related to cancer diagnosis (OR 1.97), previous bleeding (OR 7.09), hemoglobin level (OR 4.94), atrial fibrillation (OR 3.50), oral anticoagulation (OR 3.67) and renal dysfunction. Mortality and the combined secondary endpoint were associated with lower use of invasive coronary angiography and antiplatelet and neurohormonal blocker therapy. After propensity score matching (350 patients), there were no statistically significant differences in endpoints between the populations. CONCLUSION: Bleeding risk was not significant higher in the cancer population compared to patients with similar characteristics, nor were mortality or ischemic risk. The presence of cancer should not preclude simultaneous ACS treatment.

Long term follow-up of Marfan Syndrome - experience of an adult congenital heart disease centre.

BACKGROUND: Marfan Syndrome (MFS) is one of the most common connective tissue disorders. The aim of this study was to characterize an adult population with MFS and evaluate its long-term prognosis. METHODS: A retrospective analysis of adult patients with MFS followed up during the past 40 years in a tertiary congenital heart disease outpatient clinic was performed. Survival analysis was performed according to different parameters, and survival curves were compared using the log-rank test. RESULTS: A total of 62 MFS patients were followed up for a mean period of 12 years (47% male; mean age, 39 years). The baseline mean aortic root diameter (ARD) at the Valsalva sinus was 42.4 ± 10.3 mm, with 15% of patients having moderate-to-severe aortic regurgitation and seven patients with acute aortic syndrome. The Bentall procedure was the most commonly performed surgical technique, and five patients required re-operation. Of the 17 pregnancies, 29% developed fetal complications; however, there was no maternal morbidity or mortality. A total of ten deaths occurred at a mean age of 52 years. Patients with an ARD ≤ 45 mm had a significantly lower all-cause mortality rate than patients with 45 < ARD ≤ 50 mm or with ARD > 50 mm (P = 0.004 and P < 0.001, respectively). Heart failure symptoms were associated with a worse outcome (P = 0.041), while the presence of extracardiac involvement had a protective effect (P < 0.001). CONCLUSION: MFS-related aortopathy is associated with high morbidity rates. In the overall population, an ARD > 45 mm at the time of diagnosis was associated with higher mortality during follow-up.

Myocardial Work Brings New Insights into Left Ventricular Remodelling in Cardio-Oncology Patients.

Serial transthoracic echocardiographic (TTE) assessment of 2D left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) are the gold standard screening methods for cancer therapeutics-related cardiac dysfunction (CTRCD). Non-invasive left ventricular (LV) pressure-strain loop (PSL) provides a novel method of quantifying myocardial work (MW) with potential advantages to evaluate the impact of cardiotoxic treatments on heart function. We prospectively assessed breast cancer female patients undergoing cancer therapy through serial monitoring by 2D and 3D TTE. Patients were evaluated at T0, T1 and T2 (before, 4-6 and 12-14 months after starting therapy, respectively). Through PSL analysis, MW indices were calculated. A total of 122 patients, with a mean age of 54.7 years, who received treatment with anthracyclines (77.0%) and anti-HER2 (75.4%) were included. During a mean follow-up of 14.9 ± 9.3 months, LVEF and GLS were significantly diminished, and 29.5% developed CTRCD. All MW indices were significantly reduced at T1 compared with baseline and tended to return to baseline values at T2. Global work index and global work efficiency showed a more pronounced variation in patients with CTRCD. The presence of more than one cardiovascular risk factor, obesity and baseline left atrium volume were predictors of changes in MW parameters. In conclusion, breast cancer treatment was associated with LV systolic dysfunction as assessed by MW, with its peak at 4-6 months and a partial recovery afterwards. Assessment of myocardial deformation parameters allows a more detailed characterization of cardiac remodelling and could enhance patient screening and selection for cardioprotective therapeutics.

Not all pseudoaneurysms are femoral-A transcaval transcatheter aortic valve replacement rare complication.

We report a case of a 73-year-old male with multiple comorbidities, including postpoliomyelitis severe scoliosis, referred to our tertiary center due to a severe symptomatic aortic stenosis, considered high risk for surgical aortic valve replacement (AVR). Due to unsuitable femoral and subclavian accesses, the patient underwent a transcaval transcatheter AVR (TAVR) procedure, complicated by the development of an iatrogenic infrarenal aortic pseudoaneurysm with aortocaval fistula. Scoliosis can cause varying anatomic relationships between retroperitoneal vessels and intervertebral disk spaces, which increase the difficulty of the procedure and consequently lead to this vascular complication. Although most aortocaval fistulas close spontaneously after 1 year, the risk of pseudoaneurysm rupture in this critical area was crucial in the decision of a new successful percutaneous aortic stent intervention.

When "Wait and See" may be the Best Option: Two Case Reports of Left Main Artery Spontaneous Dissection.

INTRODUCTION: Spontaneous coronary artery dissection (SCAD) is frequently underdiagnosed, and the most appropriate management is unknown, particularly in high-risk anatomical subsets. CASE REPORTS: Two cases of females in their 60-70s with few cardiovascular risk factors or relevant past medical history, who presented acute myocardial infarction, have been presented. Both were submitted to invasive coronary angiography, with a suspicion of SCAD in both cases, which was later supported by coronary computed tomography angiography (CCTA). They were managed conservatively under close monitoring, with a favorable outcome. Repeated CCTA showed significant improvement, and both patients remained asymptomatic and free from complications 6 and 12 months after the initial event. CONCLUSION: The recognition of spontaneous coronary artery dissection is essential for the correct management of these cases because, unlike acute coronary syndrome due to atherosclerotic disease, the results of revascularization in those patients are suboptimal and conservative management is probably the best option, even in patients with high-risk anatomy.

Prognostic power of anaerobic threshold parameters in patients with transposition of the great arteries and systemic right ventricle.

INTRODUCTION: Both transposition of the great arteries (TGA) previously submitted to a Senning/Mustard procedure and congenitally corrected TGA (cc-TGA) have the systemic circulation supported by the morphological right ventricle, thereby rendering these patients to heart failure events risk. The aim of this study was to evaluate cardiopulmonary exercise test parameters for stratifying the risk of heart failure events in TGA patients. METHODS: Retrospective evaluation of adult TGA patients with systemic circulation supported by the morphological right ventricle submitted to cardiopulmonary exercise test in a tertiary centre. Patients were followed up for at least 1 year for the primary endpoint of cardiac death or heart failure hospitalisation. Several cardiopulmonary exercise test parameters were analysed as potential predictors of the combined endpoint and their predictive power were compared (area under the curve). RESULTS: Cardiopulmonary exercise test was performed in 44 TGA patients (8 cc-TGA), with a mean age of 35.1 ± 8.4 years. The primary endpoint was reached by 10 (22.7%) patients, with a mean follow-up of 36.7 ± 26.8 months. Heart rate at anaerobic threshold had the highest area under the curve value (0.864), followed by peak oxygen consumption (pVO2) (0.838). Heart rate at anaerobic threshold ≤95 bpm and pVO2 ≤20 ml/kg/min had a sensitivity of 87.5 and 80.0% and a specificity of 82.4 and 76.5%, respectively, for the primary outcome. CONCLUSION: Heart rate at anaerobic threshold ≤95 bpm had the highest predictive power of all cardiopulmonary exercise test parameters analysed for heart failure events in TGA patients with systemic circulation supported by the morphological right ventricle.

[Peritoneal Dialysis in the First Two Years of Life: Experience of a Nephrology and Renal Transplantation Pediatric Unit]. / Diálise Peritoneal nos Dois Primeiros Anos de Vida: Experiência de uma Unidade de Nefrologia e Transplantação Renal Pediátrica.

INTRODUCTION: Peritoneal dialysis is the dialytic method of choice in chronic end-stage renal disease in children. This study main purpose was to characterize the long-term survival of a pediatric population who began peritoneal dialysis within the first two years of life. MATERIAL AND METHODS: A descriptive and retrospective study was performed in a portuguese nephrology and renal transplantation pediatric unit, between January 1991 and August 2014. End-stage renal disease etiology, mortality, comorbidities and complications of peritoneal dialysis and end-stage renal disease, growth and psychomotor development were evaluated. RESULTS: Twenty children started peritoneal dialysis within the first two years of life. There were six deaths, but no deaths of children with primary chronic kidney disease were registered over the past decade. The 14 living children were characterized; 13 were males. Congenital abnormalities of the kidney and urinary tract were the leading etiology of chronic kidney disease (45%). The average age start of peritoneal dialysis was 6.1 months; six children started before 30 days of life. Peritonitis was the most frequent cause of hospitalization. Ten children were transplanted at an average age of 5.3 years. All of the children who are still in peritoneal dialysis have short stature, but nine of the transplanted have final height within the expected for their mid-parental height target range. Nine (64%) had some type of neurodevelopmental delay. DISCUSSION: Peritoneal dialysis is a technique possible and feasible since birth, as evidenced in the study, as more than half of children successfully started it before 6 months of life. It allows long-term survival until the possibility of renal transplantation despite the associated morbidity, including peritonitis and complications of chronic renal disease. The ten transplanted children improved their growth, recovered from chronic anemia and improved dyslipidemia, compared with the period of dialysis. However, the average waiting time until the renal transplant was 5.3 years higher than other international centers. CONCLUSION: These data support the use of peritoneal dialysis from birth, but complications and the worst growth reflect the need to develop strategies to optimize care relating to nutrition, growth and development and to reduce pre-transplant time.

Introdução: A diálise peritoneal é o método dialítico de eleição perante doença renal crónica terminal em idade pediátrica. O objetivo deste estudo foi caracterizar a sobrevivência a longo prazo de uma população de crianças, que iniciou diálise peritoneal nos dois primeiros anos de vida. Material e Métodos: Estudo descritivo e retrospetivo, realizado numa unidade de nefrologia e transplantação renal pediátrica portuguesa, no período de janeiro de 1991 a agosto de 2014. Avaliou-se etiologia da doença renal crónica terminal, mortalidade, comorbilidades e complicações da diálise peritoneal e da doença renal crónica terminal, crescimento e desenvolvimento psicomotor.Resultados: Vinte crianças iniciaram diálise peritoneal antes dos dois anos. Ocorreram seis óbitos; não houve mortalidade em crianças com doença renal primária nos últimos 10 anos. Caracterizaram-se os 14 sobreviventes, 13 do sexo masculino. As anomalias congénitas do rim e do trato urinário constituíram a principal causa de doença renal crónica terminal (45%).O início de diálise peritoneal ocorreu em média aos 6,1 meses, em seis casos antes dos 30 dias de vida. A peritonite foi o motivo mais frequente de internamento. Dez crianças foram transplantadas, com idade média de 5,3 anos. Em relação ao crescimento, as quatro crianças que se mantêm em diálise peritoneal têm baixa estatura, mas nove dos transplantados têm uma estatura final dentro do esperado para a sua estatura-alvo familiar. Nove (64%) tiveram alterações no desenvolvimento psicomotor. Discussão: A diálise peritoneal é uma técnica possível e exequível desde o nascimento, tal como evidenciado nesta amostra, em que se iniciou com sucesso em mais de metade das crianças antes dos seis meses de vida. Permite uma sobrevivência a longo prazo até à possibilidade do transplante renal apesar da morbilidade associada, nomeadamente as peritonites e as complicações da doença renal crónica. As dez crianças transplantadas desta amostra melhoraram o seu crescimento, recuperaram da anemia crónica e melhoraram da dislipidémia, comparativamente com o período em diálise. No entanto, o tempo médio de espera até ao TR de 5,3 anos foi superior ao de outros centros internacionais.Conclusão: Estes dados apoiam a utilização da diálise peritoneal desde o nascimento, embora as complicações e o pior crescimento associados reflitam a necessidade de desenvolver estratégias para otimizar nutrição, crescimento e desenvolvimento e reduzir o tempo pré-transplante renal.