Rifampin is commonly used for the treatment of tuberculosis and staphylococcal infections, as well as for prevention of infection in cardiac valve and bone surgeries. We report a case of profound hypotension after anesthesia induction with propofol in a patient who was treated with two 600 mg doses of rifampin for prophylaxis of infection before surgery. In a retrospective case-control study of 75 patients, we confirmed this potentially serious drug-drug interaction. After rifampin, there was a significant and prolonged arterial blood pressure reduction when patients received propofol, but not thiopental.

OBJECTIVE:

We assessed the impact of pregnancy on long-term cardiac outcomes in women with prior surgery for congenital pulmonary valve anomalies.

STUDY DESIGN:

Data on all reproductive age women with prior pulmonary valve repair or replacement, cared for at a tertiary institution over a 10-year period, were analyzed. Kaplan Meier curves and Proportional Hazards Models were estimated to assess the impact of pregnancy and multiparity on a composite long-term adverse outcome defined as death, heart failure, or unanticipated cardiac surgery. Peripartum cardiac complications were also assessed.

RESULTS:

Thirty-three parous and 20 nulliparous, non-pregnant controls with primary pulmonary valve replacement or repair were identified. Among the parous women, there were 95 pregnancies (median 3.0 [1-10]) resulting in 81 live births. Peripartum cardiac complications occurred in 28 (29.8%, 95% CI 20.4, 39.2) of pregnancies. A composite adverse long-term cardiac outcome occurred in 17/33 parous women, over 417 person years (4 per hundred person years) and 1/20 nulliparous women over 258 person years (0.4 per hundred person years); women with pregnancies were more likely at any point in time to have a composite long-term adverse cardiac outcome compared to nulliparous controls. Women with 2 or more pregnancies were more likely to have a composite adverse cardiac outcome than those with less than 2 pregnancies (HR 8.8, 95% CI 1.5, 50.3).

CONCLUSIONS:

Peripartum cardiac complications are common in women with prior pulmonary valve repair or replacement. Pregnancy appears to increase the risk of long-term adverse cardiac outcomes in these patients when compared to nulliparous controls.

BACKGROUND:

The Carpentier-Edwards pericardial valve was designed to minimize structural valve deterioration. Excellent durability and low incidence of valve-related complications have been reported. The objective of the present study was to analyze clinical results after 25 years of experience with this valve implanted in the aortic position. The effect of patient age at the time of surgery was also evaluated.

METHODS:

This is a retrospective cohort study of 2,405 patients from November 1981 to March 2011. Primary outcomes of interest were survival and freedom from major adverse effects such as thromboembolic, endocarditis, and reoperation.

RESULTS:

Sixty percent were male, with a mean age of 71 ± 9 years old. Actuarial survival rates including early deaths averaged 78% ± 2%, 55% ± 2%, and 16 % ± 2% after 5, 10, and 20 years of follow-up, respectively. The freedom rate of valve reoperation for prosthesis dysfunction and all other causes averaged 98 % ± 0.2%, 96% ± 1%, and 67% ± 4% at 5, 10, and 20 years. Patients younger than 60 years of age had a 15-year survival averaging 54% ± 5% compared with patients aged between 60 and 70 years of age averaging 46% ± 3% and with patients older than 70 years of age averaging 28% ± 3% (p = 0.001). Survival at 5, 10, and 20 years for patients who had concomitant CABG [coronary artery bypass grafting] were 78% ± 1%, 55% ± 2%, and 9% ± 3% compared with no concomitant CABG (84% ± 1%, 62% ± 2%, and 22% ± 3% (p < 0.001)).

CONCLUSIONS:

Carpentier-Edwards pericardial valve implantation in the aortic position is secure and durable. The effects of age influence reoperation rate and survival as well as a concomitant coronary artery bypass procedure.

OBJECTIVE:

The length of hospital stay (LOS) allows for the evaluation of the efficiency of a given hospital facility, as well as providing a basis for measuring the number of hospital beds required to provide assistance to the population in a specific area.

METHODS:

A retrospective survey was conducted on a database of 3,010 patients submitted to coronary artery bypass graft (CABG) from July, 2009 to July, 2010.

RESULTS:

Among 2,840 patients that met the inclusion criteria, 92.1% had their surgery paid by the Brazilian Unified Health System (Sistema Único de Saúde - SUS) and 7.9% by health plans or themselves (non-SUS). 70.2% were male, the average age was 61.9 years old, and the average risk score (EuroScore) was 2.9%. The SUS and the non-SUS groups did not differ regarding the waiting time for surgery (WTS) (2.59± 3.10 vs. 3,02±3,70 days for SUS and non-SUS respectively; p=0.790), but did differ with respect to the length of stay in intensive care unit (2.17±3.84 vs. 2.52±2.72 days for SUS and non-SUS respectively; p < 0.001), the postoperative period (8.34±10.32 vs. 9,19±6.97 days for SUS and non-SUS respectively; p < 0.001), and the total LOS (10.93±11.08 vs. 12.21±8.20 days for SUS and non-SUS respectively; p < 0.001). The non-SUS group had more events of non-elective surgery (p=0.002) and surgery without cardiopulmonary bypass (p=0.012). The groups did not differ regarding the associated valve procedure (p=0.057) nor other non-valve procedures (p=0.053), but they did differ with respect to associated non-cardiac procedures (p=0.017). ICU readmission (p=0.636) and postoperative complications rates were similar in both groups (p=0.055).

CONCLUSION:

The Non-SUS group showed longer LOS compared to the SUS group.

Brain natriuretic peptide (BNP) is a marker of systolic and diastolic dysfunction and a strong predictor of mortality in heart failure patients. The present study aimed to assess the relationship of BNP with aortic stenosis (AS) severity and prognosis. The cohort comprised 289 high-risk patients with severe AS who were referred for transcatheter aortic valve implantation. Patients were divided into tertiles based on BNP level: I (n = 96); II (n = 95), and III (n = 98). Group III patients were more symptomatic, had higher Society of Thoracic Surgeons and EuroSCORE scores, and had a greater prevalence of renal failure, atrial fibrillation, and previous myocardial infarction; lower ejection fraction and cardiac output; and higher pulmonary pressure and left ventricular end diastolic pressure. The degree of AS did not differ among the 3 groups. Stepwise forward multiple regression analysis identifies ejection fraction and pulmonary artery systolic pressure as independent correlates with plasma BNP. Mortality rates during a median follow-up of 319 days (range 110 to 655) were significantly lower in Group I compared with Groups II and III, p <0.001. After multivariable adjustment, the strongest correlates for mortality were renal failure (hazard ratio 1.44, p = 0.05) and medical/balloon aortic valvuloplasty (HR 2.2, p <0.001). Mean BNP decreased immediately after balloon aortic valvuloplasty from 1,595 ± 1,229 to 1,252 ± 1,076, p = 0.001 yet increased to 1,609 ± 1,264, p = 0.9 at 1 to 12 months. After surgical aortic valve replacement, there was a nonsignificant, immediate decrease in BNP level from 928 ± 1,221 to 896 ± 1,217, p = 0.77, continuing up to 12 months 533 ± 213, p = 0.08. After transcatheter aortic valve implantation, there was no significant decrease in BNP immediately after the procedure; however, at 1-year follow-up, the mean BNP level decreased significantly from 568 ± 582 to 301 ± 266 pg/dl, p = 0.03. In conclusion, a high BNP level in high-risk patients with severe AS is not an independent marker for higher mortality. BNP level does not appear to be significantly associated with the degree of AS severity but does reflect heart failure status.

Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is a lysosomal storage disorder caused by deficiency of N-acetylgalactosamine-6-sulfate sulfatase, which results in systemic accumulation of glycosaminoglycans (GAGs), keratan sulfate and chondroitin-6-sulfate. Accumulation of these GAGs causes characteristic features as disproportionate dwarfism associated with skeletal deformities, genu valgum, pigeon chest, joint laxity, and kyphoscoliosis. However, the pathological mechanism of systemic skeletal dysplasia and involvement of other tissues remain unanswered in the paucity of availability of an autopsied case and successive systemic analyses of multiple tissues. We report here a 20-year-old male autopsied case with MPS IVA, who developed characteristic skeletal features by the age of 1.5years and died of acute respiratory distress syndrome five days later after occipito-C1-C2 cervical fusion. We pathohistologically analyzed postmortem tissues including trachea, lung, thyroid, humerus, aorta, heart, liver, spleen, kidney, testes, bone marrow, and lumbar vertebrae. The postmortem tissues relevant with clinical findings demonstrated 1) systemic storage materials in multiple tissues beyond cartilage, 2) severely vacuolated and ballooned chondrocytes in trachea, humerus, vertebrae, and thyroid cartilage with disorganized extracellular matrix and poor ossification, 3) appearance of foam cells and macrophages in lung, aorta, heart valves, heart muscle, trachea, visceral organs, and bone marrow, and 4) storage of chondrotin-6-sulfate in aorta. This is the first autopsied case with MPS IVA whose multiple tissues have been analyzed pathohistologically and these pathological findings should provide a new insight into pathogenesis of MPS IVA.

OBJECTIVES:

We seek to report the results of percutaneous valve-in-valve therapy using the Melody valve (Medtronic, Minneapolis, Minnesota) for patients with degenerated mitral and tricuspid bioprosthetic valves.

BACKGROUND:

Open surgery for replacement of degenerated bioprosthetic valves is associated with morbidity and mortality.

METHODS:

Nineteen patients (median age 65 years, range 10 to 88 years; 7 males) with degenerated mitral (n = 9) or tricuspid (n = 10) bioprosthetic valves underwent transvenous valve-in-valve implantation of the Melody valve.

RESULTS:

In the mitral patients, the mean Society of Thoracic Surgeons mortality score was 13.3 ± 5.6%. All patients had a prosthetic valve mean diastolic inflow gradient ≥5 mm Hg. Moderate or worse regurgitation was present in 7 of 9 mitral and 7 of 10 tricuspid patients. Implantation of a Melody valve was successful in all. Among the mitral patients, mean diastolic gradient decreased from 12.3 ± 4.6 mm Hg to 5.2 ± 2 mm Hg (p < 0.01). Residual regurgitation was trivial to mild in 6, mild to moderate in 2, and moderate in 1 patient. Among the tricuspid patients, mean diastolic gradient decreased from 10.0 ± 4.3 mm Hg to 5.6 ± 2.5 mm Hg (p < 0.01). Residual regurgitation was trivial to mild in 9 and mild to moderate in 1 patient. New York Heart Association functional class improved in 17 of 19 patients (p < 0.01). No periprocedural deaths, myocardial infarctions, strokes, or valve embolizations occurred. Vascular access site complications occurred in 4 patients.

CONCLUSIONS:

Percutaneous valve-in-valve implantation of the Melody valve in the mitral or tricuspid position for treatment of bioprosthetic valve dysfunction is feasible and can lead to significant symptomatic improvement in carefully selected high-risk patients.

OBJECTIVES:

This study sought to report on the feasibility and early results of transcatheter aortic valve implantation employing a second-generation device in a series of patients with pure aortic regurgitation.

BACKGROUND:

Efficacy and safety of transcatheter aortic valve implantation in patients with calcific aortic stenosis and high surgical risk has been demonstrated. However, experience with implantation for severe noncalcified aortic regurgitation has been limited due to increased risk for valve dislocation or annular rupture.

METHODS:

Five patients (mean age: 66.6 ± 7 years) underwent transapical implantation of a JenaValve (JenaValve Technology GmbH, Munich, Germany) transcatheter heart valve for moderate to severe, noncalcified aortic regurgitation. All patients were considered high risk for surgical aortic valve replacement after evaluation by an interdisciplinary heart team (logistic EuroSCORE [European System for Cardiac Operative Risk Evaluation] range 3.1% to 38.9%). Procedural and acute clinical outcomes were analyzed.

RESULTS:

Implantation was successful in all cases without relevant remaining aortic regurgitation or signs of stenosis in any of the patients. No major device- or procedure-related adverse events occurred and all 5 patients were alive with improved exercise tolerance at 3-month follow-up.

CONCLUSIONS:

Noncalcified aortic regurgitation continues to be a challenging pathology for transcatheter aortic valve implantation due to the risk for insufficient anchoring of the valve stent within the aortic annulus. This report provides first evidence that the JenaValve prosthesis may be a reasonable option in these specific patients due to its unique stent design, clipping the native aortic valve leaflets, and offering promising early results.

INTRODUCTION:

Calcific aortic stenosis is the most common cardiac valve lesion and is becoming increasingly prevalent as life expectancy rises. There is evidence that patients in remote and rural areas with certain diseases have worse outcomes and present to specialist services later than their urban counterparts. It is not known whether patients with aortic stenosis follow a similar pattern. The aim of this study was to investigate whether increasing rurality was associated with later presentation to healthcare services at a more advanced stage of aortic stenosis.

METHODS:

This was a retrospective cohort study. Using ICD-10 discharge codes and local databases, 605 patients with aortic stenosis who presented between 31 November 1999 and 1 December 2008 were identified. Aortic stenosis was defined as a pressure gradient across the aortic valve of 25 mmHg or more. Patients with prior aortic valve replacement were excluded. Clinical notes were reviewed for all patients. Gender, age and pressure gradient across the aortic valve at presentation and patient GP-practice location were recorded. Patients were then assigned a Clinical Peripherality Index score based on the postcode of their GP's practice to define rurality. Patient data were compared across the six defined levels of clinical peripherality by ANOVA.

RESULTS:

Mean patient age was 73 ± 13 years, and 336 (54%) were male. The peak gradient across the valve was 41.1 ± 26.7 mmHg. There was no association between the level of clinical peripherality and the stage of aortic stenosis at presentation, age or gender (all <i>p</i> >0.05).

CONCLUSIONS:

There was no urban-rural gradient in the severity of aortic stenosis at presentation in this remote Scottish cohort. This suggests that patients with this condition in remote areas do not present later in their disease trajectory.

SETTING:

Left-sided cardiac lesions have a birth prevalence of approximately 1 in 1000 and have been shown to be heritable in pedigree studies. A large microdeletion at 16p12.1 is associated with childhood developmental delay, and initial studies describing this deletion identified left-sided lesions as an enriched phenotype compared with a control population.

OBJECTIVE:

The aim of this study is to determine whether patients with left-sided cardiac lesions have an increased frequency of 16p12.1 microdeletions as compared with control populations.

DESIGN:

A cohort of 262 probands with left-sided lesions, including 53 with isolated aortic stenosis/bicuspid aortic valve, 83 with coarctation of the aorta with or without aortic stenosis/bicuspid aortic valve, and 126 with hypoplastic left heart syndrome were assessed for copy number variation at 16p12.1. The control cohort included 595 patients with conotruncal defects as a cardiac control and 971 healthy children.

RESULTS:

We detected one patient in the left-sided lesion cohort with a large duplication partially overlapping the reported 16p12.1 microdeletion, along with one patient each in the conotruncal and control cohorts with a deletion in the same region. None of these patients had dysmorphic features, extracardiac malformations, or developmental delay.

CONCLUSION:

In our cohort, structural variation at 16p12.1 was not identified with increased frequency in patients with left-sided lesions as compared with controls.