Filipe Macedo

Dextro-transposition of the great arteries (D-TGA) is a congenital heart disease (CHD) classically palliated with atrial switch (ATR-S) and nowadays corrected with arterial switch (ART-S). Our aim was to observe a group of D-TGA patients followed in an adult CHD Outpatient Clinic. We analyzed a group of D-TGA patients born between 1974 and 2001. Adverse events were defined as a composite of death, stroke, myocardial infarction or coronary revascularization, arrhythmia, and ventricular, baffle or significative valvular dysfunction. A total of 79 patients were enrolled, 46% female, mean follow-up of 27±6 years after surgery. ATR-S was performed in 54% and ART-S in 46%; median age at procedure was 13 months and 10 days, respectively. During follow-up, almost all ART-S remained in sinus rhythm versus 64% of ATR-S (p=0.002). The latter group had a higher incidence of arrhythmias (41% versus 3%, pversus 0%, p<0.001); mean time to SVSD was 25 years. In ART-S, the most frequent complication was significant valvular regurgitation (14%). Regarding time-to-event analysis, 80% and 40% of ATR-S maintained adverse-events-free after 20 and 30 years, respectively; the time-to-first-adverse-event was 23 years and there was no difference compared to ART-S (Log-rank=0.596). ART-S tended to maintain more preserved biventricular function than ATR-S (Log-rank=0.055). After a long-term free of adverse events, ATR-S patients experienced more arrhythmias and SVSD. ART-S complications were predominantly anastomosis related; SVSD or arrhythmias were rare.

Breast cancer (BC) patients treated with anthracyclines and/or anti-HER2-targeted therapies (AHT) are highly associated with cardiovascular toxicity (CVT). Our objective was to evaluate the risk of CVT secondary to cancer treatment and the role of cardioprotective-drugs (CPD) in BC patients. We collected a retrospective cohort of females with BC treated with chemotherapy and/or AHT from 2017 to 2019. CVT was defined as LVEFvs 25.0%, p=0.006). Patients already on CPD presented a higher LVEF at 6-months follow-up (62.5% vs 59.2%, p=0.017). Patients submitted to AHT and anthracycline therapy had higher risk of developing CVT. In the AHT sub-group, pre-treatment with CPD was significantly associated with a lower prevalence of CVT. These results highlight the importance of cardio-oncology evaluation and strengthen the value of primary prevention.

Elderly people represent a vulnerable and increasing population presenting with acute coronary syndrome (ACS). Our goal was to evaluate a group of very old patients who underwent emergency coronary angiography (CA). We retrospectively analyzed a group of very old patients (≥90-year-old) who underwent emergency CA from 2008 to 2020. Survival and MACE [a composite of all-cause death, ischemic stroke, ACS or hospitalization for acute heart failure] were compared with an aged-matched control population with ACS not submitted to emergency CA. A total of 34 patients were enrolled, 56% female, median age of 92-year-old. Almost all patients had STE-ACS. In CA, 65% had multivessel disease and coronary intervention was performed in 71%. More than one-third evolved in Killip class III/IV and 70% had left ventricular dysfunction. Regarding mortality, 38% of patients died in the index event versus 25% in the aged-matched control group (p=0.319). During five years of follow-up, there was no significant difference in mortality between the two groups (Log-rank = 0.403) and more than 50% of patients died in two years. Comparing MACE occurrence, both groups were similar (Log-rank = 0.662), with more than 80% having at least one event in five years. Very-old patients submitted to emergency CA had a high rate of multivessel disease and left ventricular dysfunction, in-hospital and follow-up mortality, and MACE. Compared to an aged-matched control group not submitted to emergency CA, they showed no survival or MACE benefit during a 5-year follow-up.

Thiamine deficiency is commonly associated with malnutrition, alcoholism and bariatric surgery. Thiamine deficiency can manifest in different ways, especially in developing countries: as peripheric neuropathy, as Wernicke encephalopathy or as beriberi disease. The authors present the case of a 72-year-old male, with a hiatal hernia that led to thiamine deficiency due to malnutrition. The initial clinical manifestation was an ST-elevation myocardial infarct equivalent, an ECG with a shark-fin pattern that evolved to a Wellens type B pattern. The patient evolved with severe altered mental status. A Wernicke encephalopathy diagnosis was confirmed by MRI; the patient was medicated with high-dose thiamine, with quick recovery, both neurologic and cardiac. The clinical history and response to treatment confirm the diagnosis of Wernicke encephalopathy and beriberi disease.

Life-threatening ventricular arrhythmias (VA) may occur in patients with unknown cardiac disease. A sizable part of them remains labeled as Idiopathic VA and limited data is available regarding their natural history. Our aim was to evaluate the long-term clinical outcomes of survivors of an idiopathic life-threatening VA. Patients who survived an idiopathic life-threatening VA referred to an ICD were included and followed for a median follow-up of 7 years. Clinical and device data were collected and a comparison between genders was made. A total of 29 patients, 41% female, mean age of 50 (19) years were studied; all were implanted with an ICD at index hospitalization. At follow-up, an etiological diagnosis was established in 38% of patients. Genetic testing improved the diagnosis and allowed the identification of a distinct clinical entity in 60% of patients (p=0.04, OR = 7.0), especially in women. Regarding ICD data, 31% received appropriate therapies with a median time to first appropriate shock of 39 months (IQR 12-46 months). Men had a significantly higher prevalence of appropriated shocks (50% vs 8%, p=0.04), with a similar time to the first arrhythmic event between genders. Two of the patients died, both from non-arrhythmic causes. Etiologic diagnosis and recurrence prediction in patients with idiopathic VA is challenging, even with long-term follow-up and sophisticated diagnostic evaluation. Genetic testing significantly improved the diagnostic yield, especially in women. Arrhythmia recurrence occurred in about one-third of patients and is significantly higher in men, underscoring the importance of ICD implantation.

Background: Acute total occlusion of the unprotected left main coronary artery (ATOLMCA) is a dramatic entity with very high mortality. Owing to its infrequency, there is limited and inconsistent data regarding this population. Purpose: To describe the clinical presentation, short- and long-term outcomes of patients with ATOLMCA. Methods: This retrospective multicentric cohort study included all patients presenting with acute (<12h) myocardial infarction (MI) due to ATOLMCA (Thrombolysis In Myocardial Infarction - TIMI=0) between January 2008 and December 2020 in three tertiary hospitals. Results: In the period of the study, 11,036 emergent coronary angiographies were performed in the participating centers, 59 of which were ATOLMCA (0.5%). Mean age of patients at the time of the event was 61.2 (±12.2) years. Seventy-three percent were male. At presentation, 72.9% of patients were in cardiogenic shock, and aborted cardiac arrest occurred in 27.1%. Right dominance was present in all patients except one, who had a balanced dominance. Primary percutaneous coronary intervention (PCI) was performed in 89.8% of the patients, with angiographic success being achieved in 55.6% of the procedures. Overall, the in-hospital mortality rate was 57.6%. Mortality was significantly higher in patients without angiographic criteria for PCI success (87.5 vs 36.7%, p<0.001). Among survivors, 91.7% were still alive at 1-year and 66.7% at 5 years of follow-up. Conclusion: Patients with ATOLMCA have a dismal prognosis. Most patients present with cardiogenic shock, and a significant number develop cardiac arrest during the acute phase. Despite medical care, in-hospital mortality is high. Patients with left dominance may not even reach the hospital. Among survivors, long term outcomes are reasonable. Further studies are needed in order to improve the management and outcomes of patients with ATOLMCA. Funding Acknowledgement: Type of funding sources: None.

Aortic valvular calcium score (AVCS) is useful in patients with aortic stenosis for whom echocardiography was not conclusive in grading its severity. Transcatheter aortic valve implantation (TAVI) is an established procedure of treatment in symptomatic severe AS. The burden of aortic valve calcification has been associated with some TAVI related complications (as perivalvular leaks), but at the same time it is well accepted that some degree of calcification is needed to ensure stable anchoring of the prosthesis to the aortic annulus. To assess if there is a correlation between aortic valve calcium score and mean transvalvular gradient 6 months after TAVI – is a higher AVCS correlated with lower mean transaortic gradient after TAVI? We performed a single-center, retrospective cohort study including patients who underwent TAVI with a preoperative standardized contrast enhanced MSCT with AVCS available. Clinical and echocardiographic data were collected previously to TAVI (pre-TAVI) and at 6 months follow up (6M-FUP). A total of 187 patients were included, with 54% female and a mean age of 79.4±9.0 years old. Most patients had tricuspid aortic valve (95.7%); 5 patients had aortic bicuspidy and 3 had aortic valve bioprothesis. Concerning the valve type, 73.3% had new generation prosthesis and the main valve used was the CoreValve Evolut Pro (33.7%). Also, 38,5% needed balloon pre-dilation before TAVI. The mean pre-TAVI aortic transvalvular maximum and mean gradients were 76.5±23.2 mmHg and 48.3±15.5 mmHg, respectively; mean aortic valve area was 0.75±0.16 cm². The mean AVCS was 2851±1524 AU (Agaston Units); 81.2% of women had AVCS>1300 AU and 74.4% men had AVCS >2000 AU. Comparing transvalvular aortic gradients previously and 6M-FUP after TAVI, there was an average differential of maximum gradient of 61±22 mmHg and of mean gradient of 40±15 mmHg. A negative and weak correlation was found between the AVCS and the maximum gradient (pearson coefficient of −0.181, p=0.02) and between mean gradient at 6M-FUP (pearson coefficient of −0.191, p=0.014). AVCS is a significant predictor for death, stroke and perivalvular leaks after TAVI. On the other hand, high AVCS is associated with better seating in the native annulus during deployment. Nevertheless, high AVCS did not strongly correlated with mean transaortic gradient 6 months after TAVI. Type of funding sources: None.

Percutaneous valve commissurotomy (PMC) is a viable alternative to mitral valve (MV) surgery in the treatment of patients with clinically significant mitral stenosis (MS). About 40% of patients treated with PMC will require at least one reintervention (either PMC or MVS) along time. To evaluate the long-term results of PMC in patients with rheumatic MS. We retrospectively analysed all consecutive patients between 1991 and 2008 with clinically significant rheumatic MS undergoing PMC. Clinical and echocardiographic data were collected at baseline and during early and long-term follow-up. MACE was a composite of adverse events defined as all-cause mortality, MV re-intervention or cardiovascular hospitalization. A total of 124 patients were enrolled: 108 (87%) were female, with a mean age at the time of PMC of 46±11 years. At baseline, 34% patients were in NYHA class ≥ III and 81% had a Wilkins score ≤8; all patients had preserved biventricular systolic function and 83% presented pulmonary hypertension. Regarding associated valve disease, 46 patients had mild tricuspid regurgitation (TR), 19 mild aortic regurgitation (AR), 14 moderate IT and 5 moderated AR. Most of the procedures were successful (91%) and without complications (94%), with median improvement in MV area of 0.9 cm² (IQR 0.5) and median reductions in mean transmitral gradient (MTG) of 6 mmHg (IQR 6) and in pulmonary artery systolic pressure (PASP) of 8 mmHg (IQR 10) early after PMC. During the mean follow-up of 20±6 years, 52 (42%) of patients had MV re-intervention (86% surgery and 14% re-PMC), 37 (30%) were hospitalized and 30 (24%) died. Concerning time-to-event analysis, approximately 80% of patients kept MACE-free after 10 years; after 30 years, more than 20% continued MACE-uneventful, approximately 50% were alive and about 45% were free from re-intervention. Considering patients submitted to surgical re-intervention, 9 underwent MV valvuloplasty and the others MV replacement with mechanical (32) or biological prothesis (11). At the same procedure, 23 patients were submitted to tricuspid annuloplasty, 9 to other valve replacement and one to coronary artery bypass graft. Using Cox regression, we found that the presence of moderate disease of other valves at PMC time was associated with a 2.3-fold greater rate of re-intervention compared to patients with none or mid disease of other valves (HRcrude 2.3; 95% IC 1.221–4.331, p=0.017). After adjusting for the success of the PMC and for mitral regurgitation after PMC, the observed effect remained significant (HRadjusted = 2.7; 95% CI 1.417–5.233, p=0.003). PMC was safe and effective in clinically significant rheumatic MS. Most of the patients were free from adverse events after 10 years and half were alive after 30 years. Still, about 40% required re-intervention, with moderate disease of other valves as its independent predictor. Type of funding sources: None. Figure 1 Figure 2

Introdução O transplante cardíaco ortotópico é o tratamento atual de escolha para pacientes selecionados com insuficiência cardíaca em estágio terminal. Com o aprimoramento das técnicas cirúrgicas e a eficiência dos novos tratamentos imunossupressores, a sobrevida em curto prazo melhorou acentuadamente ao longo dos anos. No entanto, esses pacientes ainda sofrem comorbidades importantes causadas por complicações crônicas do transplante, como rejeição, doença vascular do enxerto (DVE) e malignidade. Distúrbios do ritmo são comuns em pacientes transplantados cardíacos e, em algumas circunstâncias, [...] Introdução O transplante cardíaco ortotópico é o tratamento atual de escolha para pacientes selecionados com insuficiência cardíaca em estágio terminal. Com o aprimoramento das técnicas cirúrgicas e a eficiência dos novos tratamentos imunossupressores, a sobrevida em curto prazo melhorou acentuadamente ao longo dos anos. No entanto, esses pacientes ainda sofrem comorbidades importantes causadas por complicações crônicas do transplante, como rejeição, doença vascular do enxerto (DVE) e malignidade. Distúrbios do ritmo são comuns em pacientes transplantados cardíacos e, em algumas circunstâncias,