Peripheral extracorporeal membrane oxygenation system as salvage treatment of patients with refractory cardiogenic shock: preliminary outcome evaluation.

The novel Permanent Life Support (PLS; Maquet, Jostra Medizintechnik AG, Hirrlingen, Germany) as peripheral veno-arterial extracorporeal membrane oxygenation (ECMO) support system has been investigated as treatment for patients with refractory cardiogenic shock (CS). Between January 2007 and July 2011, 73 consecutive adult patients were supported on peripheral PLS ECMO system at our institution (55 men; age 60.3 ± 11.6 years, range: 23-84 years). Indications for support were failure to wean from cardiopulmonary bypass in the setting of postcardiotomy (n = 50) and primary donor graft failure (n = 8), post-acute myocardial infarction CS (n = 12), and CS on chronic heart failure (n = 3). Mean support time was 10.9 ± 7.6 days (range: 2-34 days). Overall, 26 (35.6%) patients died on ECMO. Among survivors on ECMO, 44 (60.2%) patients were successfully weaned from support, and three (4.1%) were switched to a mid-long-term ventricular assist device. Thirty-three (45.2%) were successfully discharged. The following variables were significantly different if survivors and nonsurvivors on ECMO were compared: age (P = 0.04), female gender (P < 0.01), cardiopulmonary resuscitation before ECMO (P < 0.01), lactate level before ECMO (P = 0.01), number of platelets, fresh frozen plasma units, and packed red blood cells (PRBCs) transfused during ECMO support (P = 0.03, P = 0.02, and P < 0.01), blood lactate level (P = 0.01), and creatine kinase isoenzyme MB (CK-MB) relative index 72 h after ECMO initiation (P < 0.001), and multiple organ failure on ECMO (P < 0.01). Stepwise logistic regression identified blood lactate level and CK-MB relative index at 72 h after ECMO initiation, and number of PRBCs transfused on ECMO as significant predictors of mortality on ECMO (P = 0.011, odds ratio [OR] = 2.48; 95% confidence interval [CI] = 1.11-3.12; P = 0.012, OR = 2.81, 95% CI = 1.026-2.531; and P = 0.012, OR = 1.94, 95% CI = 1.02-5.21; respectively). Patients with an initial poor hemodynamic status could benefit by rapid peripheral installation of PLS ECMO. The blood lactate level, CK-MB relative index, and PRBCs transfused should be strictly monitored during ECMO support.

Novel compound mutations in the mitochondrial translation elongation factor (TSFM) gene cause severe cardiomyopathy with myocardial fibro-adipose replacement.

Primary mitochondrial dysfunction is an under-appreciated cause of cardiomyopathy, especially when cardiac symptoms are the unique or prevalent manifestation of disease. Here, we report an unusual presentation of mitochondrial cardiomyopathy, with dilated phenotype and pathologic evidence of biventricular fibro-adipose replacement, in a 33-year old woman who underwent cardiac transplant. Whole exome sequencing revealed two novel compound heterozygous variants in the TSFM gene, coding for the mitochondrial translation elongation factor EF-Ts. This protein participates in the elongation step of mitochondrial translation by binding and stabilizing the translation elongation factor Tu (EF-Tu). Bioinformatics analysis predicted a destabilization of the EF-Ts variants complex with EF-Tu, in agreement with the dramatic steady-state level reduction of both proteins in the clinically affected myocardium, which demonstrated a combined respiratory chain enzyme deficiency. In patient fibroblasts, the decrease of EF-Ts was paralleled by up-regulation of EF-Tu and induction of genes involved in mitochondrial biogenesis, along with increased expression of respiratory chain subunits and normal oxygen consumption rate. Our report extends the current picture of morphologic phenotypes associated with mitochondrial cardiomyopathies and confirms the heart as a main target of TSFM dysfunction. The compensatory response detected in patient fibroblasts might explain the tissue-specific expression of TSFM-associated disease.

Impaired mitochondrial biogenesis is a common feature to myocardial hypertrophy and end-stage ischemic heart failure.

Mitochondrial (mt) DNA depletion and oxidative mtDNA damage have been implicated in the process of pathological cardiac remodeling. Whether these features are present in the early phase of maladaptive cardiac remodeling, that is, during compensated cardiac hypertrophy, is still unknown. We compared the morphologic and molecular features of mt biogenesis and markers of oxidative stress in human heart from adult subjects with compensated hypertrophic cardiomyopathy and heart failure. We have shown that mtDNA depletion is a constant feature of both conditions. A quantitative loss of mtDNA content was associated with significant down-regulation of selected modulators of mt biogenesis and decreased expression of proteins involved in mtDNA maintenance. Interestingly, mtDNA depletion characterized also the end-stage phase of cardiomyopathies due to a primary mtDNA defect. Oxidative stress damage was detected only in failing myocardium.

Aortic valve replacement in a patient with ostegenesis imperfecta A case report.

AIM: Osteogenesis imperfecta (OI) is an inherited connective tissue disorder in which fragile bones readily cause fractures. Aortic root dilatation, aortic valve regurgitation and mitral valve prolapse are uncommon cardiovascular manifestations of OI. Cardiac surgery in these patients carries a high risk of complications due to increased tissue and capillary fragility. We describe an open heart surgery in a woman with isolated aortic valve regurgitation secondary to OI. MATERIAL OF STUDY: A 58-year-old woman was referred to our hospital for surgical correction of aortic valve regurgitation. She had a past history of recurrent long bone fractures, and OI was diagnosed in the childhood. A standard median sternotomy was performed; the sternum was found to be thin and brittle. The native aortic valve was replaced with a size 23 mm stented aortic bioprosthesis. The sternum was closed with stainless steel wires. RESULTS: The postoperative course was uneventful, and the patient was discharged home on the eighth postoperative day. We used thoracic band to avoid sternal diastasis. One year postoperatively, the echocardiogram showed a normal aortic bioprosthesis function without paravalvular leakage. The sternum was stable without dehiscence. DISCUSSION: The mortality rate in cardiac surgery patients with heritable generalized connective tissue disorders, such as osteogenesis imperfecta, is high. Although tissue friability had no impact on surgical outcome, it should be kept in mind when operating on patients with OI. CONCLUSIONS: We highlight the importance of a meticulous surgical technique, together with a strategy for management of anticipated perioperative complications to ensure a successful outcome. KEY WORDS: Aortic valve, Endocardirtis, Mitral valve, Replacement.

Levitronix CentriMag third-generation magnetically levitated continuous flow pump as bridge to solution.

The Levitronix CentriMag (Levitronix LLC, Waltham, MA) ventricular assist device (VAD) is a magnetically levitated rotary pump designed for temporary extracorporeal support. Between February 2004 and May 2010, 42 consecutive adult patients were supported with Levitronix at our institution (32 men; age 62.3 ± 10.5 years, range: 31-76 years). Indications for support were (group A, n = 37) failure to wean from the cardiopulmonary bypass in the setting of postcardiotomy (n = 23), primary donor graft failure (n = 4), or right ventricular failure after axial left VAD (LVAD) placement (n = 10) and (group B, n = 5) refractory heart failure after acute myocardial infarction. The mean support time was 11.2 ± 6.8 days (range: 3-43 days) in group A and 8.6 ± 4.3 days (range: 5-11 days) in group B. In the postcardiotomy cohort (group A), 11 (47.8%) patients were weaned from support as all were supported graft failure patients. Eight patients of axial LVAD cohort were weaned from right VAD (RVAD). One patient was bridged to heart transplantation (Htx). Thirteen (35.1%) patients died on support in group A. In group B, one patient was bridged to Htx and four died on support. In overall population, bleeding requiring reoperation occurred in 15 (35.7%) cases and cerebral major events in four (9.5%). There were no device failures. Of the 23 (54.7%) patients who recovered and were discharged home, 20 (47.6%) are presently alive, and additionally, two patients of both groups who were bridged to Htx (overall n = 22, 52.3%). The Levitronix proved to be useful in patients previously considered nonsuitable for transplantation or long-term assist device. The device was technically easy to manage, and the results were encouraging.

Echocardiographic follow-up after implanting 17-mm Regent mechanical prostheses.

The aim of this study was to evaluate midterm echocardiographic results and changes in quality of life after aortic valve replacement with 17-mm St. Jude Medical Regent mechanical prostheses in patients with aortic valve stenosis. The study population was 34 women and 2 men, aged 31-83 years. Echocardiographic follow-up was 100% complete at 4.1 +/- 1.8 years. Hospital mortality was 5.6%. Actuarial 5-year survival was 88.5% +/- 0.067%. Postoperative echocardiography showed significant regression of left ventricular mass index and significant reductions of peak gradient, mean gradient and mean effective orifice area index. All survivors were interviewed using the 36-item Short Form Health Survey questionnaire. Scores obtained in 7 of the 8 domains of the test were significantly higher than preoperative values. In our experience, implantation of this prosthesis allowed regression of left ventricular mass index and improved the perceived quality of life.