Effect of Pectus Excavatum on Survival

Abstract

Source: Kelly RE Jr., Lawson ML, Paidas CN, et al. Pectus excavatum in a 112-year autopsy series: anatomic findings and the effect on survival. J Pediatr Surg. 2005;40:1275–1278.The purpose of this study was to determine the frequency of pectus excavatum and associated conditions (like recurvatum and funnel chest) in a large autopsy series. These authors retrospectively identified patients with pectus excavatum at autopsy from a single hospital database at Johns Hopkins. Each case was matched with 4 race- and sex-matched patients, 2 autopsied immediately before and 2 immediately after the target patient. The authors reviewed 50,496 autopsy files from 1889 to 2001 in which 62 cases of pectus excavatum were identified, the earliest in 1947.Eighteen patients died before the age of 1 year, 6 between 1 and 4 years, 0 between 5 and 14 years, 21 between 14 and 65 years, and 17 at or beyond 65 years. Of 21 patients who died between ages 14 and 65 years, 2 had Marfan syndrome, and there were single cases with Ehlers-Danlos syndrome, Noonan syndrome, Duchenne muscular dystrophy, and Rett syndrome. One patient had scleroderma, another was suspected of having scleroderma, and 1 patient developed severe scoliosis even after undergoing pectus repair at age 2.Overall analysis indicates that patients with pectus excavatum did not live as long as the race- and sex-matched controls: median age at death in the pectus excavatum group was 33 years compared to 44 years for controls. Further statistical analysis indicated that 63% of patients survived to 40 years compared to 71% of the controls (P<.05). On the other hand, 22% of patients surviving beyond 56 years survived to 70 years, while only 17% of the controls lived to that age (P<.05). Speculating about the reason for survival in this subgroup was beyond the scope of the article.Patients with pectus excavatum who survived to 65 years did not have coexisting conditions identified at autopsy. Coexisting diagnoses were noted in 9 of 21 (43%) patients who died when between the ages of 14 and 65 years and in 5 of 18 (28%) patients who died before age 1 year. Recent data have also suggested pectus excavatum may affect cardiovascular function through compression of the heart, and surgical repair may reverse the negative cardiovascular effects of this malformation.1 The authors conclude that repair of pectus excavatum before adulthood has the potential to improve longevity, most notably in patients with coexisting conditions.Dr. Cavett has disclosed no financial relationships relevant to this commentary.Pectus excavatum is a common condition that can frequently be found in conjunction with connective tissue disorders as well as acquired cardiovascular abnormalities. The authors of this article admit that an autopsy study is subject to some severe limitations, including the inability of such a study to determine the severity of the pectus defect. The authors argue that this study design – a fixed cohort study in which the exposure (pectus) is permanent and easily identifiable and there is no loss of the cohort to follow-up – should reliably identify whether pectus excavatum affects survival.The article illustrates how little was written about pectus excavatum before the mid-1940s, the likely explanation for no reported cases of pectus abnormalities in the first 20,400 autopsies reviewed. Recent success in minimally invasive pectus repair using thoracoscopically guided placement of a 2-mm-thick alloy bar of variable length placed through lateral thoracic incisions has greatly altered the morbidity of the surgical repair.2The cardiopulmonary effects of pectus excavatum are only now coming to light. Recent data indicate that chest wall compression on the right ventricle may be a source of the frequent complaint of exercise intolerance.3 It is possible that chest wall impingement on the right ventricle and atrium might influence survival in these patients. Surgical repair has also recently been reported to offer relief of positional angina caused by compression on the right coronary artery by the pectus defect.1 Our experience suggests that right ventricular compression (and in some cases right atrial compression) is common and limits filling enough that cardiac output should be limited at exercise levels.Does repair of pectus excavatum in the pediatric age group lead to extended lifespan? One would expect that to be the case, but we do not yet have the evidence to prove it.