Mitral Valve Surgery for Rheumatic Lesions in Young Patients

BACKGROUND: The appropriateness of rheumatic mitral valve repair remains controversial due to the risks of recurrent mitral dysfunction and need for reoperation. The aims of this study were to determine the overall short- and long-term outcomes of pediatric rheumatic mitral valve surgery in our center. METHODS: Single-center, observational, retrospective study that analyzed the results of rheumatic mitral valve surgery in young patients, consecutively operated by the same team, between 1999 and 2014. RESULTS: We included 116 patients (mean age = 12.6 ± 3.5 years), of which 66 (57%) were females. A total of 116 primary surgical interventions and 22 reoperations were performed. Primary valve repair was possible in 86 (74%) patients and valve replacement occurred in 30 (26%). Sixty percent of the patients were followed up beyond three months after surgery (median follow-up time = 9.2 months [minimum = 10 days; maximum = 15 years]). Long-term clinical outcomes were favorable, with most patients in New York Heart Association functional class I (89.6%) and in sinus rhythm (85%). Freedom from reoperation for primary valve repair at six months, five years, and ten years was 96.4% ± 0.25%, 72% ± 0.72%, and 44.7% ± 1.34%, respectively. Freedom from reoperation for primary valve replacement at six months, five years, and ten years was 100%, 91.7% ± 0.86%, and 91.7% ± 0.86%, respectively. Mitral stenosis as the primary lesion dictated early reintervention. CONCLUSIONS: Despite the greater rate of reoperation, especially when the primary lesion was mitral stenosis, rheumatic mitral valve repair provides similar clinical outcomes as compared with replacement, with the advantage of avoiding anticoagulation.

Expressão de Aterosclerose Sub-Clínica para Diferentes Factores de Risco Cardiovasculares em Populações Jovens

INTRODUCTION: Carotid intima-media thickness (cIMT) is considered an early marker for atherosclerosis, but there are few studies on the expression of this marker in younger populations. OBJECTIVES: To evaluate cIMT in younge patients (aged 30-50 years) and its expression according to cardiovascular risk factors. METHODS: We analyzed individuals admitted for an invasive cardiac procedure. Normal cIMT was defined as < 0.90 mm, thickened as 0.90-1.50 mm and atherosclerotic plaque as > 1.50 mm. Lipid profile, anthropometric parameters, fasting blood glucose and estimated GFR were also determined. RESULTS: A total of 106 patients were included (59% male), with a mean age of 43 +/- 5 years, 36% with hypertension, 22% smokers, 32% with known hyperlipidemia, 16% with diabetes, 39% under statin therapy and 40% with metabolic syndrome (AHA/NHLBI definition). Mean cIMT was 0.69 +/- 0.26 mm, and was normal in 74% of the patients, thickened in 20% and with atherosclerotic plaques in 6%. cIMT correlated directly with age (r = 0.26, p = 0.007), log fasting glucose (r = 0.21, p = 0.04), and log triglycerides (r = 0.24, p = 0.017), and tended to correlate with the number of components of metabolic syndrome (r = 0.17, p = 0.08). However, on multivariate analysis, only age remained as an independent predictor (r = 0.29, p = 0.005). Diabetic patients had greater cIMT (0.81 +/- 0.22 vs. 0.67 +/- 0.26 mm, p = 0.039) and there was a trend for greater cIMT in those with metabolic syndrome (0.75 +/- 0.29 vs. 0.66 +/- 0.23 mm, p = 0.09). There were no differences for the other risk factors, A higher number of risk factors in a single patient showed a trend for increased cIMT (p = 0.083) CONCLUSIONS: Age is the only independent determinant of cIMT in a young population. Diabetic patients have greater cIMT and a trend was seen in those with metabolic syndrome, possibly influenced by its relation with diabetes, one of the components of the metabolic syndrome.

Operação de Ross: Resultados a Médio Prazo

The Ross procedure has been used in children and young adults for aortic valve replacement and the correction of complex obstruction syndromes of the left ventricular outflow tract. We report the mid-term results of the Ross procedure in a single institution and performed by the same surgical team. Population: Between March 1999 and December 2005, 18 patients were operated on using the Ross procedure. The mean age at the time of surgery was 12 years, being 12 patients male (67%). The primary indication for surgery was isolated aortic valve disease, being the predominant abnormality in 58% of cases aortic regurgitation and in 42% left ventricular outflow tract obstruction. Associated lesions included sub-aortic membrane in 3 patients (16%), small VSD in 2 patients (11%), bicuspid aortic valve in 4 patients (22%) and severe left ventricular dysfunction and mitral valve regurgitation in 1 patient (6%). Ten of the 18 patients (56%) had been submitted to previous surgical procedures or percutaneous interventions. Results: Early post-operative mortality was not seen, but two patients (11%), had late deaths, one due to endocarditis, a year after the Ross procedure, and the other due to dilated cardiomiopathy and mitral regurgitation. The shortest time of follow-up is 6 months and the longest 72 months (median 38 months). Of the 16 survivors, 14 patients are in class I of the NYHA and 2 in class II, without significant residual lesions or need for re-intervention. The 12 patients with more than a year of follow up revealed normal coronary perfusion in all patients and no segmental wall motion abnormalities. Nevertheless, two of the 12 patients developed residual dynamic obstruction of LVOT and in three patients aortic regurgitation of a mild to moderate degree was evident. Significant gradients were not verified in the RVOT. Conclusions: The Ross procedure, despite its complexity, can be undertaken with excellent immediate results. Aspects such as the dilation of the neo aortic root and homograft evolution can not be considered in a study of this nature, seeing that the mean follow up time does not exceed 5 years.

De Novo Hypertension and Decline of Renal Function in a Young Woman with Systemic Lupus Erythematosus and Antiphospholipid Syndrome

Antiphospholipid syndrome nephropathy and lupus nephritis have similar clinical and laboratory manifestations and achieving the accuracy of diagnosis required for correct treatment frequently necessitates a kidney biopsy. We report the case of a 29-year-old woman referred to the nephrology service for de novo hypertension, decline of renal function and proteinuria. She had had systemic lupus erythematosus and antiphospholipid syndrome since the age of 21 and was taking oral anticoagulation. Two weeks later, after treatment of hypertension and achievement of adequate coagulation parameters, a percutaneous renal biopsy was performed. The biopsy revealed chronic lesions of focal cortical atrophy, arterial fibrous intimal hyperplasia and arterial thromboses, which are typical features of antiphospholipid syndrome nephropathy. We describe the clinical manifestations and histopathology of antiphospholipid syndrome nephropathy and review the literature on renal biopsy in patients receiving anticoagulation.

ABO-Incompatible Liver Transplantation in Acute Liver Failure: A Single Portuguese Center Study

INTRODUCTION: ABO-incompatible liver transplantation (ABOi LT) is considered to be a rescue option in emergency transplantation. Herein, we have reported our experience with ABOi LT including long-term survival and major complications in these situations. PATIENT AND METHODS: ABOi LT was performed in cases of severe hepatic failure with imminent death. The standard immunosuppression consisted of basiliximab, corticosteroids, tacrolimus, and mycophenolate mofetil. Pretransplantation patients with anti-ABO titers above 16 underwent plasmapheresis. If the titer was above 128, intravenous immunoglobulin (IVIG) was added at the end of plasmapheresis. The therapeutic approach was based on the clinical situation, hepatic function, and titer evolution. A rapid increase in titer required five consecutive plasmapheresis sessions followed by administration of IVIG, and at the end of the fifth session, rituximab. RESULTS: From January 2009 to July 2012, 10 patients, including 4 men and 6 women of mean age 47.8 years (range, 29 to 64 years), underwent ABOi LT. At a mean follow-up of 19.6 months (range, 2 days to 39 months), 5 patients are alive including 4 with their original grafts. One patient was retransplanted at 9 months. Major complications were infections, which were responsible for 3 deaths due to multiorgan septic failure (2 during the first month); rejection episodes (4 biopsy-proven of humoral rejections in 3 patients and 1 cellular rejection) and biliary. CONCLUSION: The use of ABOi LT as a life-saving procedure is justifiable in emergencies when no other donor is available. With careful recipient selection close monitoring of hemagglutinins and specific immunosuppression we have obtained acceptable outcomes.

Safety and Effectiveness of the Genous™ Endothelial Progenitor Cell-Capture Stent in the First Year Following ST-Elevation Acute Myocardial Infarction: A Single Center Experience and Review of the Literature

PURPOSE: The Genous™ stent (GS) is designed to accelerate endothelization, which is potentially useful in the pro-thrombotic environment of ST-elevation acute myocardial infarction (STEMI). We aimed to evaluate the safety and effectiveness of the GS in the first year following primary percutaneous coronary intervention (PCI) and to compare our results with the few previously published studies. METHODS AND MATERIALS: All patients admitted to a single center due to STEMI that underwent primary PCI using exclusively GS, between May 2006 and January 2012, were enrolled. The primary study endpoints were major adverse cardiac events (MACEs), defined as the composite of cardiac death, acute myocardial infarction and target vessel revascularization, at one and 12months. RESULTS: In the cohort of 109 patients (73.4% male, 59 ±12years), 24.8% were diabetic. PCI was performed in 116 lesions with angiographic success in 99.1%, using 148 GS with median diameter of 3.00mm (2.50-4.00) and median length of 15mm (9-33). Cumulative MACEs were 2.8% at one month and 6.4% at 12months. Three stent thromboses (2.8%), all subacute, and one stent restenosis (0.9%) occurred. These accounted for the four target vessel revascularizations (3.7%). At 12months, 33.9% of patients were not on dual antiplatelet therapy. CONCLUSIONS: GS was safe and effective in the first year following primary PCI in STEMI, with an apparently safer profile comparing with the previously published data. SUMMARY: We report the safety and effectiveness of the Genous™ stent (GS) in the first year following primary percutaneous coronary intervention in ST-elevation acute myocardial infarction. A comprehensive review of the few studies that have been published on this subject was included and some suggest a less safe profile of the GS. Our results and the critical review included may add information and reinforce the safety and effectiveness of the GS in ST-elevation in acute myocardial infarction.

Hereditary Neuropathy with Liability to Pressure Palsy Presenting with Hand Drop in a Young Child

Hereditary neuropathy with liability to pressure palsy (HNPP) results from the deletion of the PMP22 gene in chromosome 17p11.2. Clinically, it presents with painless pressure palsies, typically in the 2nd and 3rd decades of life, being a rare entity in childhood. We present the case study of a six-year-old male child who presented with left hand drop that he kept for over four weeks. Electrophysiological studies suggested HNPP and genetic studies confirmed it. With this paper, we pretend to create awareness to this entity as a diagnosis to be considered in a child with painless monoparesis and to emphasize the importance of electrophysiological studies in the diagnosis.

Psychiatric and Psychosocial Predictors of Medical Outcome After Liver Transplantation: a Prospective, Single-Center Study

OBJECTIVE: Recognizing the potential impact of psychiatric and psychosocial factors on liver transplant patient outcomes is essential to apply special follow-up for more vulnerable patients. The aim of this article was to investigate the psychiatric and psychosocial factors predicted medical outcomes of liver transplanted patients. METHODS: We studied 150 consecutive transplant candidates, attending our outpatient transplantation clinic, including 84 who had been grafted 11 of whom died and 3 retransplanted. RESULTS: We observed that active coping was an important predictor of length of stay after liver transplantation. Neuroticism and social support were important predictors of mortality after liver transplantation. CONCLUSION: It may be useful to identify patients with low scores for active coping and for social support and high scores for neuroticism to design special modes of follow-up to improve their medical outcomes.

Prophylactic Use of Liposomal Amphotericin B in Preventing Fungal Infections Early After Liver Transplantation: a Retrospective, Single-Center Study

In this study the authors evaluated the efficacy of prophylaxis with liposomal amphotericin B (L-AmB) in the incidence of fungal infections (FI) during the first 3 months after liver transplant (LT). The study was retrospective and accessed a 4-year period from 2008 to 2011. All patients who died in the first 48 hours after LT were excluded. Patients were divided by the risk groups for FI: Group 1, high-risk (at least 1 of the following conditions: urgent LT; serum creatinine >2 mg/dL; early acute kidney injury [AKI] after LT; retransplantation; surgical exploration early post-LT; transfused cellular blood components [>40 U]); and Group 2, low-risk patients. Group 1 patients were further separated into those who received antifungal prophylaxis with L-AmB and those who did not. Prophylaxis with L-AmB consisted of intravenous administration of L-AmB, 100 mg daily for 14 days. Four hundred ninety-two patients underwent LT; 31 died in the first 48 hours after LT. From the remaining 461 patients, 104 presented with high-risk factors for FI (Group 1); of these, 66 patients received antifungal prophylaxis and 38 did not. In this group 8 FI were observed, 5 in patients without antifungal prophylaxis (P = .011). Three more FI were identified in Group 2. By logistic regression analysis, the categorical variable high-risk group was independently related to the occurrence of invasive FI (P = .006). We conclude that prophylaxis with L-AmB after LT was effective in reducing the incidence of FI. No influence on mortality was detected.

The Hybrid Approach for Palliation of Hypoplastic Left Heart Syndrome: Intermediate Results of a Single-Center Experience

INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is a major cause of cardiac death during the first week of life. The hybrid approach is a reliable, reproducible treatment option for patients with HLHS. Herein we report our results using this approach, focusing on its efficacy, safety and late outcome. METHODS: We reviewed prospectively collected data on patients treated for HLHS using a hybrid approach between July 2007 and September 2014. RESULTS: Nine patients had a stage 1 hybrid procedure, with seven undergoing a comprehensive stage 2 procedure. One patient completed the Fontan procedure. Five patients underwent balloon atrial septostomy after the hybrid procedure; in three patients, a stent was placed across the atrial septum. There were three deaths: two early after the hybrid procedure and one early after stage two palliation. Overall survival was 66%. CONCLUSIONS: In our single-center series, the hybrid approach for HLHS yields intermediate results comparable to those of the Norwood strategy. The existence of dedicated teams for the diagnosis and management of these patients, preferably in high-volume centers, is of major importance in this condition.

Liver Depurative Techniques: A Single Liver Transplantation Center Experience

In a liver transplant (LT) center, treatments with Prometheus were evaluated. The main outcome considered was 1 and 6 months survival. Methods. During the study period, 74 patients underwent treatment with Prometheus; 64 were enrolled,with a mean age of 51 13 years; 47men underwent 212 treatments (mean, 3.02 per patient). The parameters evaluated were age, sex, laboratorial (liver enzymes, ammonia) and clinical (model for end-stage liver disease and Child-Turcotte-Pugh score) data. Results. Death was verified in 23 patients (35.9%) during the hospitalization period, 20 patients (31.3%) were submitted to liver transplantation, and 21 were discharged. LT was performed in 4 patients with acute liver failure (ALF, 23.7%), in 7 patients with acute on chronic liver failure (AoCLF, 43.7%), and in 6 patients with liver disease after LT (30%). Seven patients who underwent LT died (35%). In the multivariate analysis, older age (P ¼ .015), higher international normalized ratio (INR) (P ¼ .019), and acute liver failure (P ¼ .039) were independently associated with an adverse 1-month clinical outcome. On the other hand, older age (P ¼ .011) and acute kidney injury (P ¼ .031) at presentation were both related to worse 6-month outcome. For patients with ALF and AoCLF we did not observe the same differences. Conclusions. In this cohort, older age was the most important parameter defining 1- and 6-month survival, although higher INR and presence of ALF were important for 1-month survival and AKI for 6-month survival. No difference was observed between patients who underwent LT or did not have LT.

Infections After Liver Transplantation: A Retrospective, Single-Center Study

Objective. To access the incidence of infectious problems after liver transplantation (LT). Design. A retrospective, single-center study. Materials and Methods. Patients undergoing LT from January 2008 to December 2011 were considered. Exclusion criterion was death occurring in the first 48 hours after LT. We determined the site of infection and the bacterial isolates and collected and compared recipient’s variables, graft variables, surgical data, post-LT clinical data. Results. Of the 492 patients who underwent LT and the 463 considered for this study, 190 (Group 1, 41%) developed at least 1 infection, with 298 infections detected. Of these, 189 microorganisms were isolated, 81 (51%) gram-positive bacteria (most frequently Staphylococcus spp). Biliary infections were more frequent (mean time of 160.4 167.7 days after LT); from 3 months after LT, gram-negative bacteria were observed (57%). Patients with infections after LT presented lower aminotransferase levels, but higher requirements in blood transfusions, intraoperative vasopressors, hemodialysis, and hospital stay. Operative and cold ischemia times were similar. Conclusion. We found a 41% incidence of all infections in a 2-year follow-up after LT. Gram-positive bacteria were more frequent isolated; however, negative bacteria were commonly isolated later. Clinical data after LT were more relevant for the development of infections. Donors’ variables should be considered in future analyses.