Quality-of-life impact of diaphragm plication in patients with diaphragmatic paralysis: A retrospective study.

OBJECTIVES: While the overall incidence and prevalence of diaphragmatic paralysis are unknown due to a wide variety of underlying causes, symptomatic patients experience a marked decline in their quality of life. The goal of this study was to measure the impact of diaphragm plication surgery on the quality of life in patients who were diagnosed with diaphragmatic paralysis. METHODS: A retrospective review of the medical records of 46 patients who underwent diaphragmatic plication surgery was performed. The review included patients who experienced unilateral and bilateral diaphragmatic paralysis. Patients who underwent repeat diaphragm plication surgery were also included in the study. Patients from the retrospective cohort were then contacted by telephone to answer the Dyspnea-12 (D-12) questionnaire. Patients were asked to recall the severity of their symptoms and quality of life preplication, 1-month postplication, and 6-month postplication. Severity of symptoms was ranked as either none, mild, moderate, or severe. Values were then assigned to each rank as follows: none = 0, mild = 1, moderate = 2, and severe = 3. Relative change and statistical significance were calculated with preplication measurements used as the baseline. Scores between preplication versus 1-month postplication and 6-month postplication were then compared by Student's paired t-test. All tests were two-sided and statistical significance was set at P < 0.05. RESULTS: Forty-six patients were included in the study, from which 21 answered the D-12 questionnaire. Average scores from each component of the D-12 questionnaire showed improvement in the severity of symptoms from preplication to 1-month postplication. The latter period was then followed by continued improvement in all areas when symptoms 6-month postplication were assessed. CONCLUSION: In patients with diaphragmatic paralysis, diaphragm plication was effective in reducing patients' symptoms while improving overall quality of life.

Surgical repair of anomalous coronary arteries arising from the opposite sinus of Valsalva in infants and children.

BACKGROUND: Unroofing of anomalous coronary artery originating from the opposite sinus of Valsalva has become the procedure of choice for this congenital lesion, with surgery performed in children as young as two years old. An increasing number of this anomaly is diagnosed in infancy with no clear indication whether surgical repair should be done in this age group. This paper reviews our experience with this anomaly, and focuses on its surgical management in infants. METHODS: Between April 2002 and February 2007, eight patients underwent surgical repair of anomalous coronary artery arising from the opposite sinus of Valsalva and coursing between the aorta and pulmonary artery. Patients' age varied from two months to 28 years with a mean of 11.7 +/- 11.1 years. SURGICAL TECHNIQUE: Surgical repair involved unroofing the intramural segment of the anomalous coronary artery using cardiopulmonary bypass. RESULTS: Two patients were younger than one year (Group A), and six patients were older than one year (group B). The mean intensive care unit stay was 2.5 +/- 0.7 days for Group A and 2.8 +/- 1.9 for Group B. The mean hospital stay was 4 +/- 1.4 days for Group A and 4.3 +/- 2.4 days for Group B. There was no mortality and no complications. The mean follow-up period is 14 +/- 15.7 months with a range of one to 39 months. At the time of the last follow-up, all patients were asymptomatic in New York Heart Association class I and follow-up echocardiography on six of eight patients showed wide open coronary ostium. CONCLUSION: Unroofing the anomalous coronary artery arising from the opposite sinus of valsalva can be done in infants with minimal morbidity and mortality. Longer follow-up is needed to assess long-term results.

Recent developments in antithrombotic therapy: will sodium warfarin be a drug of the past?

Warfarin and heparin have formed the mainstay in the prophylaxis of deep vein thrombosis (DVT), stroke prevention in atrial fibrillation, and treatment of thromboembolic disease (TED). However, these choices are hampered by difficult administration, interactions with other medications, side effect profile, and limited indications for treatment. Anti-factor Xa (anti-Xa) inhibitors have already entered the drug market with the drug Fondaparinux being the first anti-Xa inhibitor to be approved for use in the U.S. by the Food and Drug Administration (FDA), and other drugs such as idraparinux being currently in development. A new class of medications, known as direct thrombin inhibitors (DTI), includes the parental agents lepirudin, argatroban and bivalirudin which have been approved by the FDA and the oral agents ximelagatran, melagatran and dabigatran. The latter three drugs which are oral DTIs may soon replace warfarin and heparin as the preferred medications for DVT prophylaxis and for reducing the relative risk of stroke. These drugs do not rely on blocking serine proteases nor do they require a co-factor (antithrombin III) like unfractionated heparin (UFH) or low molecular weight heparin (LMWH). DTIs are rapid in onset, easy to administer, do not interact with other medications or foods, have limited side effects, and can be administered in a fixed dose. The DTI ximelagatran has already been approved in several European and Asian countries, and over a dozen randomized clinical trials have been conducted demonstrating its performance to be on par with warfarin. However, approval by the FDA in the U.S. remains pending in view of reported incidences of elevations in hepatic enzymes that are currently under evaluation. This review examines the role of DTIs in the prevention and treatment of TED and the recent patents reported in the literature.

Outcomes of lung transplantation in patients with scleroderma.

Patients with pulmonary insufficiency due to scleroderma have long been considered suboptimal candidates for lung transplantation. This has been supported by small single-center experiences that did not reflect the entire U.S. experience. We sought to evaluate the outcome of patients with scleroderma who underwent lung transplantation. We conducted a retrospective review of 47 patients with scleroderma who underwent lung transplantation at 23 U.S. centers between 1987 and 2004 and were reported to the United Network for Organ Sharing. Women constituted 57% of the patients. The mean age was 46 years. Twenty-seven patients received single lung transplants (57%), and the remaining received double lung transplants. The mean cold ischemia time was 4.1 hours. There were 7 early deaths (< or =30 days) and 17 late deaths (> 30 days). The causes of early death were primary graft failure and a cardiac event in two patients each and bacterial infection and stroke in one patient each. Late mortality was due to infection in seven patients, respiratory failure in three, malignancy in two, and multisystem organ failure, rejection, pulmonary hypertension, and a cardiac event in one patient each. The causes of early and late death were not recorded for two patients. One patient received a second transplant owing to graft failure of the first. Twenty-three patients (49%) were alive at a mean follow-up of 24 months. The Kaplan-Meier 1- and 3-year survival rates were 67.6% and 45.9% respectively, which are not significantly different from those of 10,070 patients given transplants for other lung conditions during the same period (75.5% and 58.8% respectively, P = 0.25). Donor gender, recipient's age, and type of transplant did not affect survival. In carefully selected patients with scleroderma who have end-stage lung disease, lung transplantation is a valid life-saving therapeutic option. Available data suggest acceptable short-term morbidity and mortality and a long-term survival similar to that of patients given transplants for other lung conditions.

Sirolimus-associated interstitial pneumonitis in solid organ transplant recipients.

Sirolimus is a potent immunosuppressive agent used with increasing frequency in solid organ transplantation (SOT). However, it has been associated with rare but devastating pulmonary toxicity. We describe a case of pulmonary toxicity associated with the use of sirolimus in a 64-yr-old heart transplant recipient. We also review all reported cases of sirolimus-associated lung toxicity among SOT recipients in an effort to better understand the pathophysiology, risk factors, and outcomes of this rare but serious complication. A total of 64 cases have been reported since January 2000 including the present case. These consisted of 52 kidney, four lung, three liver, three heart, one heart-lung and one islet cell transplants. In most cases, patients presented with a constellation of symptoms consisting of fever, dyspnea, fatigue, cough, and occasionally hemoptysis. Although the risk factors for this association have not been clearly established, high dose, late exposure to the drug and male gender have been noticed among most. In almost all of the reported cases, sirolimus was added later in the course of immunosuppressive therapy, usually in an effort to attenuate the nephrotoxic effects of a previous regimen containing a calcineurin inhibitor. There were three deaths (4.8%) among 62 patients with known status at follow up; all deaths were among heart transplant recipients. Most patients (95%) resolved their clinical and radiographic findings with discontinuation or dose-reduction of the drug. Sirolimus-induced pulmonary toxicity is a rare but serious entity that should be considered in the differential diagnosis of a transplant recipient presenting with respiratory compromise. Dose-reduction or discontinuation of the drug can be life saving.