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Ventilatory support in facioscapulohumeral muscular dystrophy.

Respiratory insufficiency due to respiratory muscle weakness is a common complication of many neuromuscular diseases. The prevalence of respiratory failure in facioscapulohumeral muscular dystrophy (FSHD) is unknown. The authors identified 10 FSHD patients on nocturnal ventilatory support at home, representing approximately 1% of the Dutch FSHD population. Severe muscle disease, wheelchair dependency, and kyphoscoliosis appeared to be risk factors for respiratory failure.

Adult↗

A comparison of epidural bupivacaine-fentanyl and bupivacaine-clonidine in children undergoing the Nuss procedure.

The administration of epidural opioids, though effective for producing analgesia, has severe side effects in most patients. It is unknown whether clonidine can effectively replace opioids and cause fewer side effects. We compared, in this randomized trial, the incidence of vomiting and pruritus as well as the analgesic profile of three different combinations of bupivacaine, fentanyl, and clonidine administered epidurally in patients undergoing the Nuss procedure: bupivacaine + fentanyl, bupivacaine + clonidine, bupivacaine + fentanyl + clonidine. The incidence of side effects was significantly less in the bupivacaine + clonidine group (33%) compared with the bupivacaine + fentanyl (92%) and bupivacaine + fentanyl + clonidine (73%) groups (P = 0.004). Quality of postoperative analgesia was similar in the three groups. No significant complications were observed. In conclusion, clonidine is an effective and safe alternative to epidural opioids.

Adolescent↗

Surgical treatment of annulo-aortic ectasia.

Surgical interventions for annulo-aortic ectasia were analyzed and the following conclusions were obtained: 1) In the treatment of annulo-aortic ectasia, Bentall operation is not always the first choice, and satisfactory result can be achieved by either aortic valve replacement (AVR) plus tube graft replacement or AVR plus aneurysmorrhaphy. 2) Bentall operation is indicated to the cases with a) Marfan syndrome, b) aneurysm with thin wall and c) aortic regurgitation from dissection. 3) In the technique using composite graft, wrapping with aneurysmal wall is not always necessary and sinus-shaped composite graft is an advantage to prosthetic valve motion, reducing tension around the coronary artery anastomosis. 4) Aorto-coronary bypass should be combined with Bentall operation in the cases with coronary ostium involved by dissection and displaced coronary ostium.

Adult↗

Correction of pectus excavatum with a self-retaining seagull wing prosthesis. Long-term follow-up.

Between June 1958 and December 1991, 315 patients (217 male and 98 female, mean age = 17.8 +/- 5.5 years) affected by pectus excavatum (PE) were surgically treated. Most of the patients required operation for aesthetic reasons only (299 patients; 95 percent). The grade of PE (Chin classification) was I in 72 patients, II in 152, and III in the remaining 91. The surgical technique consisted of a double transversal sternotomy at the level of the lowest and highest part of the depression associated with a longitudinal sternotomy. A wedge resection of the ribs was then performed and the sternum was fixed using a stainless steel strut molded into a seagull wing prosthesis. The strut was removed 12 months postoperatively. There were no operative mortalities. Four patients had sternal wound infection that was successfully treated. The mean follow-up was 15.8 years per patient and was 60 percent complete. From the aesthetic point of view, the postoperative results were excellent in 246 patients (78 percent), good in 57 (18 percent), and poor in 12 (4 percent). All subjective symptoms, when present, disappeared after surgery. The seagull wing prosthesis appears to be safe, easy to implant and to remove, and comfortable for the patient. This technique has shown good long-term results independently of type of deformity and patient age.

Adolescent↗

Ventilatory and cardiovascular responses to exercise in patients with pectus excavatum.

PURPOSE: Uncertainty exists as to whether pectus excavatum causes true physiologic impairments to exercise performance as opposed to lack of fitness due to reluctance to exercise. The purpose of this study was to examine the effect of pectus excavatum on ventilatory and cardiovascular responses to incremental exercise in physically active patients. METHODS: Twenty-one patients with pectus excavatum (age range, 13 to 50 years; mean [+/- SD] age, 23.6 +/- 8.9 years; severity index range, 3.7 to 8.0; mean severity index, 5.1 +/- 1.2) were referred for preoperative evaluation. Eighteen of the patients (85%) had a history of performing aerobic activity ranging from 30 min to 2 h per day (mean duration, 1.0 +/- 0.61 h per day) for 3 +/- 1.5 days per week. Patients performed pulmonary function tests, and submaximal and maximal incremental exercise testing. RESULTS: On maximal exercise testing, the maximum oxygen uptake (O(2)max), and oxygen-pulse were significantly lower than the reference values (t(20) = 6.17 [p < 0.0001] and t(20) = 4.52 [p < 0.0001], respectively). Furthermore, patients exhibited cardiovascular limitation, but not ventilatory limitation. Despite their high level of habitual exercise activity, the overall metabolic threshold for lactate accumulation was abnormally low (ie, 41% of the reference value for O(2)max) especially in those with a pectus severity index (PSI) of > 4.0 (39% of the reference value of O(2)max), which is consistent with cardiovascular impairment rather than physical deconditioning. Patients with a PSI of > 4.0 were also eight times more likely to have reduced aerobic capacity than patients who had a low severity index, despite their level of exercise participation. On submaximal testing, we found that the time constant for O(2) uptake kinetics was 37.4 s for the on-transit and 41.6 s for the off-transit. The observed values for FVC, FEV(1), maximum voluntary ventilation, and diffusing capacity of the lung for carbon monoxide were significantly lower than reference values, but those for total lung capacity and residual volume were not significantly lower than reference values. CONCLUSIONS: The information derived from this study supports the opinion that pectus excavatum is associated with true physiologic impairment and reduced exercise capacity, predominantly due to impaired cardiovascular performance rather than ventilatory limitation. Furthermore, the impairment is not explained by physical deconditioning.

Adolescent↗

Cardiovascular function following surgical repair of pectus excavatum: a metaanalysis.

BACKGROUND: Despite numerous published reports, there is no consensus in the literature as to whether the surgical repair of the pectus excavatum improves cardiovascular function. As a result, it has been suggested that correction should be considered a cosmetic procedure, and therefore, many health insurance companies have questioned whether the repair of the pectus excavatum improves cardiovascular function and thus are reluctant to authorize the procedure. The purpose of this study was to apply metaanalysis methodology to generate a quantitative synthesis of the effects of surgical repair on cardiovascular function and to test the hypothesis that surgical repair of the pectus excavatum results in significant improvements in cardiovascular function. METHODS: Studies were retrieved via computerized literature searches, cross-referencing from original and review articles, and a review of the reference list by a recognized authority in the area of pectus excavatum repair. The inclusion criteria were as follows: (1) reporting quantitative measures of preoperative and postoperative cardiovascular function; (2) published in the English language; (3) indexed between January 1960 and May 2005; (4) reporting the duration between which preoperative and postoperative assessments were conducted; and (5) describing the cardiovascular assessment procedures. RESULTS: A comprehensive search of the literature identified eight studies that met all of the inclusion criteria. These studies, representing 169 pectus excavatum patients, were used for the metaanalysis. Random-effects modeling yielded a mean weighted effect size (ES) for cardiovascular function that was statistically significant (ES, 0.59; 95% confidence interval, 0.25 to 0.92; p = 0.0006). CONCLUSIONS: The findings of the present study indicated that surgical repair of the pectus excavatum significantly improves cardiovascular function and contradicts arguments that surgical repair is primarily cosmetic yielding minimal physiologic improvement.

Cardiovascular System↗

Pectus excavatum.

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Funnel Chest↗

A new plastic operation for pectus excavatum: sternal turnover surgical procedure with preserved internal mammary vessels.

The so-called turnover operation for pectus excavatum in older age groups has been disappointing due to postoperative complication such as fistual formation with necrosis of bones and muscles. Thus, the need of preserved vascular pedicle to the sternum was stressed in this type of operation. A new sternal crossover operation keeping the bilateral internal mammary vessels preserved was developed and satisfactorily applied on three clinical cases. The results indicate that this method is a useful sternal crossover procedure for patients over 15 years of age.

Adult↗

Minimally invasive concomitant cardiac procedures and repair of pectus excavatum: case report.

A minimally invasive concomitant repair of an atrial-septal defect II with patent ductus arteriosus and pectus excavatum, using a substernal steel bar was successfully done in a 10-year-old girl. Postoperative outcomes and cosmetic appearance were excellent. Therefore, a simultaneous repair of pectus excavatum with cardiac lesions performed in a minimally invasive way is feasible and should be considered, particularly in female children.

Cardiac Surgical Procedures↗

Swallowing induced supraventricular ectopics in a patient with straight back syndrome.

A 45 year old man presented with the complaint of palpitations on swallowing. Clinical and radiological examination showed features of straight back syndrome and pectus excavatum. Electrocardiogram revealed supraventricular ectopics each time the patient swallowed. This illustrates a unique case of swallowing-related rhythm disturbance.

Deglutition↗