Perforated esophageal intervention focus (PERF) study: a multi-center examination of contemporary treatment.

The treatment of esophageal perforation (EP) remains a significant clinical challenge. While a number of investigators have previously documented efficient approaches, these were mostly single-center experiences reported prior to the introduction of newer technologies: specifically endoluminal stents. This study was designed to document contemporary practice in the diagnosis and management of EP at multiple institutions around the world and includes early clinical outcomes. A five-year (2009-2013) multicenter retrospective review of management and outcomes for patients with thoracic or abdominal esophageal perforation was conducted. Demographics, etiology, diagnostic modalities, treatments, subsequent early outcomes as well as morbidity and mortality were captured and analyzed. During the study period, 199 patients from 10 centers in the United States, Canada, and Europe were identified. Mechanisms of perforation included Boerhaave syndrome (60, 30.1%), iatrogenic injury (65, 32.6%), and penetrating trauma (25, 12.6%). Perforation was isolated to the thoracic segment alone in 124 (62.3%), with 62 (31.2%) involving the thoracoabdominal esophagus. Mean perforation length was 2.5 cm. Observation was selected as initial management in 65 (32.7%), with only two failures. Direct operative intervention was initial management in 65 patients (32.6%), while 29 (14.6%) underwent esophageal stent coverage. Compared to operative intervention, esophageal stent patients were significantly more likely to be older (61.3 vs. 48.3 years old, P < 0.001) and have sustained iatrogenic mechanisms of esophageal perforation (48.3% vs.15.4%). Secondary intervention requirement for patients with perforation was 33.7% overall (66). Complications included sepsis (56, 28.1%), pneumonia (34, 17.1%) and multi-organ failure (23, 11.6%). Overall mortality was 15.1% (30). In contemporary practice, diagnostic and management approaches to esophageal perforation vary widely. Despite the introduction of endoluminal strategies, it continues to carry a high risk of mortality, morbidity, and need for secondary intervention. A concerted multi-institutional, prospectively collected database is ideal for further investigation.

Pathogenic FBN1 variants in familial thoracic aortic aneurysms and dissections.

Marfan syndrome (MFS) due to mutations in FBN1 is a known cause of thoracic aortic aneurysms and acute aortic dissections (TAAD) associated with pleiotropic manifestations. Genetic predisposition to TAAD can also be inherited in families in the absence of syndromic features, termed familial TAAD (FTAAD), and several causative genes have been identified to date. FBN1 mutations can also be identified in FTAAD families, but the frequency of these mutations has not been established. We performed exome sequencing of 183 FTAAD families and identified pathogenic FBN1 variants in five (2.7%) of these families. We also identified eight additional FBN1 rare variants that could not be unequivocally classified as disease-causing in six families. FBN1 sequencing should be considered in individuals with FTAAD even without significant systemic features of MFS.

Operative outcomes using a side-branched thoracoabdominal aortic graft (STAG) for thoraco-abdominal aortic repair.

BACKGROUND: Pre-manufactured branched grafts now allow an endovascular approach to the repair of thoraco-abdominal aortic aneurysm (TAAA) with visceral vessels' involvement. Similar grafts have been employed in open surgery, generally as a second choice for TAAAs, which are not amenable to patch/inclusion technique for visceral vessel attachment. Results with branched grafts have not been reported in series of open TAAA repairs. The purpose of this report is to describe perioperative risks and outcomes in a consecutive series of patients with pre-manufactured side-branched thoracoabdominal aortic grafts (STAGs) for surgical TAAA repair. METHODS: Between 1996 and 2009, pre-manufactured STAGs were used in 50 patients with TAAA that required reattachment of the visceral and renal arteries. Operative details, perioperative mortality and ischaemic complications were examined. RESULTS: Mean age was 53 years; 18 patients were females. The cases included redo (n = 24), patients affected by genetic disorder (Marfan) (n = 20) and patients with aortic dissection (n = 27). The mean clamp time was 84.1 min. Perioperative mortality was 12.0% (6/50). Neurologic deficits occurred in 2% (1/50). Postoperative renal dysfunction was detected in 19 patients (38%). CONCLUSION: The use of a STAG produced acceptable mortality, bowel and neurological ischaemic risks. Improved strategies to prevent renal ischaemia before and during repair of TAAA with visceral involvement are needed.

Serum myoglobin and renal morbidity and mortality following thoracic and thoraco-abdominal aortic repair: does rhabdomyolysis play a role?

OBJECTIVES: The intractability of renal dysfunction following thoracic and thoraco-abdominal aortic repair leads us to believe that the accepted mechanisms of renal injury - ischaemia and embolism - are incompletely explanatory. We studied postoperative myoglobinaemia and renal dysfunction following aortic surgery. METHODS: Between September 2006 and February 2008, we studied serum myoglobin in 109 patients requiring thoracic/thoraco-abdominal repair for three postoperative days. Forty-two of the 109 (38%) patients were female. The median age was 67 years (range 23-84 years). As we have focussed more attention on renal function, our independent renal consultants have dialysed more aggressively. We divided dialysis into: (1) creatinine indication, (2) non-creatinine indication and (3) no dialysis. RESULTS: Thirteen of the 109 (12%) patients met creatinine indication for dialysis (>4 mg dl(-1)) and an additional 28 (26%) were dialysed for other reasons. Overall mortality was 12 out of 109 (11%) cases: 11 out of 41 (27%) in dialysed patients and one out of 68 (1.5%) in non-dialysed patients. Mortality did not differ between the indications for dialysis. Predictors of mortality were baseline glomerular filtration rate (GFR), postoperative myoglobin and dialysis. The only predictor of dialysis was postoperative myoglobin. CONCLUSION: A strong relationship between postoperative serum myoglobin and renal failure suggests a rhabdomyolysis-like contributing aetiology following thoraco-abdominal aortic repair. We postulate a novel mechanism of renal injury for which mitigation strategies should be developed.

Thoracic aortic aneurysms.

Remarkable progress has been made in the surgical treatment of thoracoabdominal aortic aneurysms. The decline in mortality and complication rates can be attributed to improvements in perioperative care and in surgical technique, particularly the adoption of adjunct distal aortic perfusion and cerebrospinal fluid drainage. Neurologic deficit is no longer a major threat to patients, as the use of adjuncts has brought the incidence down to 2.4% for all thoracoabdominal aortic aneurysms. However, we continue to pursue research to improve organ preservation, particularly for the most troublesome extent II thoracoabdominal aortic aneurysm.

Staged repair of extensive aortic aneurysms: morbidity and mortality in the elephant trunk technique.

BACKGROUND: Extensive aortic aneurysms (ascending aorta, aortic arch, and descending or thoracoabdominal aorta) require innovative surgical techniques. Some surgeons advocate a single procedure with long periods of profound hypothermia, whereas others use a staged approach. We adopted a two-staged procedure (elephant trunk technique) in 1991 for elective repair of extensive aortic aneurysms. METHODS AND RESULTS: Between February 1991 and May 2000, we performed a total of 1146 aortic aneurysm operations. Of these, 182 (15.9%) operations were first- or second-stage elephant trunk procedures, performed in a total of 117 patients. Stage 1 was completed in all 117 patients. Stage 2 was completed in 65 (55.6%) of 117 patients. Thirty-day mortality rate for the first stage was 5.1% (6 of 117). Mortality rate during the interval between operations was 3.6% (4 of 111), of which 75% (3 of 4) were the result of aneurysm rupture. Thirty-day mortality rate for the second stage was 6.2% (4 of 65). A total of 43 patients did not return for second-stage repair. Among these patients, within an average period of 3.4 years (range, 1.5 months to 4.9 years), 13 of 43 (30.2%) died, 4 of 13 (30.8%) as the result of rupture. Two of 117 (1.7%) first-stage patients had postoperative stroke. No spinal cord dysfunction occurred in second-stage patients. CONCLUSIONS: Extensive aortic aneurysms can be repaired with acceptable morbidity and mortality rates through the use of the elephant trunk technique. Death was most commonly the result of rupture, both in interval patients awaiting scheduled second-stage repair and in patients who did not return. After the first stage, prompt treatment of the remaining segment is crucial to the success of staged repair.

Contemporary management of blunt aortic trauma.

Blunt thoracic aortic injury (BTAI) remains a common cause of death following blunt mechanisms of trauma. Among patients who survive to reach hospital care, significant advances in diagnosis and treatment afford previously unattainable survival. The Society for Vascular Surgery (SVS) guidelines provide current best-evidence suggestions for treatment of BTAI. However, several key areas of controversy regarding optimal BTAI care remain. These include the refinement of selection criteria, timing for treatment and the need for long-term follow-up data. In addition, the advent of the Aortic Trauma Foundation (ATF) represents an important development in collaborative research in this field.

Measurement of aortic root size by biplane angiography before cardiac valve replacement.

Aortic valve replacement (AVR) in the patient with a small aortic root demands special consideration because the hemodynamic function of artificial valves with a small external diameter is often poor. In this study, the internal diameter of the aortic root was measured from biplane ventriculography. This measured root diameter was then used to predict the external diameter of the artificial valve. Twelve patients underwent biplane ventriculography followed by AVR with Carpentier-Edwards bioprostheses. The artificial valve diameter was predicted with a correlation coefficient of 0.93, a standard error of estimate of 0.89 mm, and an average absolute difference between preoperative measurement and valve diameter of 0.69 mm. Therefore, the aortic root diameter can be accurately measured from the ventriculogram, thus detecting the patient with a small aortic root before surgery.

Dissecting aortic aneurysm involving a right-sided aortic arch.

In this case, the first reported instance of aortic dissection involving a right-sided arch, an anomalous fourth arch vessel, the left subclavian artery, arose from a congenital aortic diverticulum. This report emphasizes the need for precise anatomic definition with aortography to permit appropriate therapy when congenital anomalies of the aortic arch are complicated by dissecting hematoma.

Hemodynamic evaluation of the Carpentier-Edwards bioprosthesis in the aortic position.

The Carpentier-Edwards bioprosthesis is a glutaraldehyde-fixed porcine xenograft with a fully flexible thin-walled stent. Cardiac catheterization studies were performed in 17 patients to evaluate use of this valve in the aortic position. Hemodynamic studies established a mean peak gradient across the prosthesis of 19 mm Hg (range 5 to 65). The mean effective orifice area was calculated to be 1.6 cm2 (range 0.8 to 3.3). All patients demonstrated an improvement in functional class after operation. Mean left ventricular ejection fraction increased from 51 +/- 16 to 68 +/- 9 percent (P less than 0.004) in eight patients operated on for aortic stenosis, but was not significantly changed in patients operated on for aortic insufficiency. Hemodynamic comparison of the Carpentier-Edwards bioprosthesis with the standard Hancock xenograft showed similar effective orifice areas for the 23 and 25 mm diameter valves. In two patients studied the 21 mm Carpentier valve demonstrated a greater effective orifice area than that previously reported for the standard Hancock xenograft. The Carpentier-Edwards bioprosthesis affords both clinical and hemodynamic improvement when used in the aortic position and may allow improved effective orifice area when used in the smaller aortic root.

Traumatic injuries of the diaphragm.

Traumatic injury of the diaphragm is not an infrequent occurrence. With the rise in violence and increasing use of automobiles, more diaphragmetic injuries may be seen, especially in inner-city hospitals. Sixty-six cases from our institution within the last five years were reviewed. Of these there were 41 penetrating injuries and 23 secondary to blunt trauma. Two cases were surgically induced following a difficult decortication for pleuropulmonary tuberculosis. There were ten deaths (15 percent mortality). All deaths were related to the severity of associated injuries. In addition, we analyzed 307 patients with multiple injuries who were dead on arrival and were autopsied by the county medical examiners in a 24-month period. Of the 307 autopsied cases, 16 (5.2 percent) had ruptured diaphragms. Interestingly, all but one of these cases were associated with thoracic aortic injuries. Diagnoses of penetrating diaphragmatic injuries were made during exploration of other injuries. In blunt diaphragmatic rupture, a high index of suspicion in most important in the diagnosis. In 10 of 23 blunt injuries, visceral herniation was noted on initial x-ray films. In four, follow-up films several hours to a day later showed loops of bowel in the chest. In nine cases, there were no apparent visceral herniations on initial films, and in these, the diagnosis was made during surgery for other indications. The surgical approach to diaphragmatic injuries is individualized. Acute left-sided injuries are best approached through the abdomen. Acute right-sided injuries and all chronic injuries should be approached through the chest.

Tuberculous aneurysms of the descending thoracic aorta: report of a case with fatal rupture.

Tuberculous aortic aneurysm is a rare disease entity. The majority of affected patients succumb to perforation and exsanguination. The only chance for survival and cure is by resection and prolonged antituberculosis chemotherapy. Our case illustrates the high risk of rupture of tuberculous aortic aneurysms. Post-mortem examination revealed that the mechanism of aneurysm formation was by direct caseous involvement of the descending thoracic aorta from a juxtaposed left upper lobe parenchymal tuberculous process. Our findings also favor the concept that miliary dissemination (in the presence of tuberculous aortic aneurysm) is the result rather than the cause of the tuberculous aortic process.

The effect of mannitol and dopamine on the renal response to thoracic aortic cross-clamping.

Postoperative renal failure and insufficiency are important complications of operations that require thoracic aortic cross-clamping. Successful application of pharmacologic methods to protect renal function would be clinically useful. The ability of mannitol and dopamine to prevent renal dysfunction in a canine model of thoracic aortic cross-clamping was studied. Twenty animals were divided into four equal groups, and all underwent thoracic aortic cross-clamping for 60 minutes. An intra-aortic infusion of saline (control), mannitol, dopamine, or mannitol plus dopamine was started before, and continued during, the period of aortic occlusion. Glomerular filtration rate was significantly depressed 60 minutes after clamp release, and although there was some recovery in treated animals 150 minutes after clamp release, it remained significantly decreased (52% to 73% of baseline values, p less than 0.01). Renal blood flow was significantly reduced 60 minutes after clamp release, and there was no recovery in any group at 150 minutes (38% to 56% of baseline values, p less than 0.01). No significant differences in osmolar clearance or fractional excretion of sodium were evident between groups. These data reveal that the profound reductions in glomerular filtration and renal blood flow induced by thoracic aortic cross-clamping were not attenuated by mannitol or dopamine and suggest that efforts to protect renal function should be directed toward improving renal blood flow in the post-clamp period.

Postpneumonectomy alveolar growth does not normalize hemodynamic and mechanical function.

Immature foxhounds underwent 55% lung resection by right pneumonectomy (n = 5) or thoracotomy without pneumonectomy (Sham, n = 6) at 2 mo of age. Cardiopulmonary function was measured during treadmill exercise on reaching maturity 1 yr later. In pneumonectomized animals compared with Sham animals, maximal oxygen uptake, ventilatory response, and cardiac output during exercise were normal. Arterial and mixed venous blood gases and arteriovenous oxygen extraction during exercise were also normal. Mean pulmonary arterial pressure and resistance were elevated at a given cardiac output. Dynamic ventilatory power requirement was also significantly elevated at a given minute ventilation. These long-term hemodynamic and mechanical abnormalities are in direct contrast to the normal pulmonary gas exchange during exercise in these same pneumonectomized animals reported elsewhere (S. Takeda, C. C. W. Hsia, E. Wagner, M. Ramanathan, A. S. Estrera, and E. R. Weibel. J. Appl. Physiol. 86: 1301-1310, 1999). Functional compensation was superior in animals pneumonectomized as puppies than as adults. These data indicate a limited structural response of conducting airways and extra-alveolar pulmonary blood vessels to pneumonectomy and suggest the development of other sources of adaptation such as those involving the heart and respiratory muscles.

In vivo assessment of changes in air and tissue volumes after pneumonectomy.

We examined the progression and topographical distribution of postpneumonectomy volume changes in immature foxhounds undergoing right pneumonectomy (R-Pnx, n = 5) or sham pneumonectomy (Sham, n = 6) at 2 mo of age and subsequently raised to maturity. Volumes of lung air (Vair) and tissue (Vti) were estimated by computerized tomography (CT) scan at 7, 22, and 52 wk after surgery at a transpulmonary pressure of 20 cmH2O. Estimates of Vti by CT scan included both septal tissue as well as nonseptal tissue (small- and medium-sized airways and blood vessels); these were compared with estimates of septal Vti by an acetylene rebreathing (Rb) method. We found significant correlations between these techniques (Vair(CT) = 0.83 Vair(Rb) + 275, R = 0.97; Vti(CT) = 1.62 Vti(Rb) - 30, R = 0.81). Extravascular septal Vti returned to normal 7 wk after R-Pnx and remained normal up to maturity. Nonseptal Vti remained significantly below normal. The greatest increase in Vti occurred in the midlung region just cephalad and caudal to the heart. After an early period of accelerated tissue growth after R-Pnx, the rate of septal tissue growth matched that of somatic growth, whereas nonseptal tissue growth lagged behind. Compensatory growth of the remaining left lung was not associated with selective alterations in thoracic development.

Temporal course of gas exchange and mechanical compensation after right pneumonectomy in immature dogs.

To determine the temporal progression and magnitude of functional compensation in immature dogs raised to maturity after extensive lung resection, we performed right pneumonectomy (R-Pnx) or right thoracotomy without pneumonectomy (Sham) in matched foxhounds at 2 mo of age. At 4, 8, 20, 40, and 60 wk after surgery, static transpulmonary pressure-lung volume relationships were determined. Lung diffusing capacity, membrane diffusing capacity, pulmonary capillary blood volume, pulmonary blood flow, septal lung tissue volume, and lung volumes were measured simultaneously by a rebreathing technique. During maturation, total lung capacity, lung volume at a given distending pressure, and compliance were lower in the R-Pnx group than in the Sham group (P < 0.05). Pulmonary viscous resistance at maturity was elevated after R-Pnx. There were no significant differences in total lung diffusing capacity, membrane diffusing capacity, pulmonary capillary blood volume, pulmonary blood flow, and septal lung tissue volume between groups. Compensation of alveolar-capillary gas exchange was complete by 4-8 wk after R-Pnx, but compensation of mechanical properties remained incomplete throughout maturation. Relative magnitude of compensation after R-Pnx was greater in immature dogs than in adult dogs studied previously by similar techniques.

Compensatory alveolar growth normalizes gas-exchange function in immature dogs after pneumonectomy.

To determine the extent and sources of adaptive response in gas-exchange to major lung resection during somatic maturation, immature male foxhounds underwent right pneumonectomy (R-Pnx, n = 5) or right thoracotomy without pneumonectomy (Sham, n = 6) at 2 mo of age. One year after surgery, exercise capacity and pulmonary gas-exchange were determined during treadmill exercise. Lung diffusing capacity (DL) and cardiac output were measured by a rebreathing technique. In animals after R-Pnx, maximal O2 uptake, lung volume, arterial blood gases, and DL during exercise were completely normal. Postmortem morphometric analysis 18 mo after R-Pnx (n = 3) showed a vigorous compensatory increase in alveolar septal tissue volume involving all cellular compartments of the septum compared with the control lung; as a result, alveolar-capillary surface areas and DL estimated by morphometry were restored to normal. In both groups, estimates of DL by the morphometric method agreed closely with estimates obtained by the physiological method during peak exercise. These data show that extensive lung resection in immature dogs stimulates a vigorous compensatory growth of alveolar tissue in excess of maturational lung growth, resulting in complete normalization of aerobic capacity and gas-exchange function at maturity.

Preventing mediastinal shift after pneumonectomy does not abolish physiological compensation.

To determine the role of mediastinal shift after pneumonectomy (PNX) on compensatory responses, we performed right PNX in adult dogs and replaced the resected lung with a custom-shaped inflatable silicone prosthesis. Prosthesis was inflated (Inf) to prevent mediastinal shift, or deflated (Def), allowing mediastinal shift to occur. Thoracic, lung air, and tissue volumes were measured by computerized tomography scan. Lung diffusing capacities for carbon monoxide (DL(CO)) and its components, membrane diffusing capacity for carbon monoxide (Dm(CO)) and capillary blood volume (Vc), were measured at rest and during exercise by a rebreathing technique. In the Inf group, lung air volume was significantly smaller than in Def group; however, the lung became elongated and expanded by 20% via caudal displacement of the left hemidiaphragm. Consequently, rib cage volume was similar, but total thoracic volume was higher in the Inf group. Extravascular septal tissue volume was not different between groups. At a given pulmonary blood flow, DL(CO) and Dm(CO) were significantly lower in the Inf group, but Vc was similar. In one dog, delayed mediastinal shift occurred 9 mo after PNX; both lung volume and DL(CO) progressively increased over the subsequent 3 mo. We conclude that preventing mediastinal shift after PNX impairs recruitment of diffusing capacity but does not abolish expansion of the remaining lung or the compensatory increase in extravascular septal tissue volume.

Effect of pneumonectomy on the remaining lung in dogs.

To determine the magnitude of functional compensation after pneumonectomy and whether compensation is related to maturity of the animal at the time of resection, we performed left pneumonectomy in either adult or 10-wk-old beagles. Studies were performed in adults 7-9 mo after surgery and in puppies 18-23 mo after surgery when the dogs reached full maturity. Results were compared with those in age- and sex-matched unoperated controls. Measurements included pressure-volume relationships, pulmonary hemodynamics, rebreathing studies of lung volume, diffusing capacity and its components, lung tissue volume, and pulmonary blood flow. Computerized-tomographic scans were performed in the puppy groups to determine changes in thoracic shape and size. Morphometric analysis of the lungs was performed under light microscopy. There was partial compensation for loss of one lung by functional improvement in the remaining lung. Compensation was greater in those pneumonectomized as puppies than as adults. Volume of the remaining lung was larger than predicted for a given transpulmonary pressure in both groups. Diffusing capacity, pulmonary capillary blood volume, and lung tissue volume were larger than expected for the normal right lung. After pneumonectomy, compliance of the rib cage was greater in puppies than in adults. Weight of the costal diaphragm was reduced in pneumonectomized puppies. Pulmonary hypertension at rest did not develop, and pulmonary vascular reactivity to hypoxia was unchanged after pneumonectomy in both groups. Significant correlations were obtained between physiological and morphometric measurements.

Corrosive burns of the esophagus and stomach: a recommendation for an aggressive surgical approach.

During a six-year period ending in December, 1980, 62 patients with a history or clinical evidence of corrosive ingestion were admitted into our institutions. The majority were adults who had attempted suicide. Strong alkali (lye), the most common corrosive agent involved, was ingested by more than half of the patients (39). The remaining 23 patients had ingested weak alkali or nonalkali corrosive agents. Of the 27 patients with severe esophagogastric burns (second- and third-degree), a 43.5% incidence overall, liquid lye was responsible in 21, including 7 of 8 patients with extensive full-thickness esophagogastric necrosis. In sharp contrast, only 1 of the 23 patients who had ingested weak alkali or nonalkali corrosive agents had serious esophagogastric injury. In the first two years of this review, the management approach was the so-called standard one (esophagoscopy, steroids, antibiotics, and dilation) (Group 1). The results were disappointing. In 5 of 9 patients with endoscopic findings of second-degree burns, stricture requiring dilation developed, and all 4 with extensive full-thickness esophagogastric necrosis died. In contrast, during the last four years, with the adoption of a more aggressive surgical approach, that is, early surgical intervention including the use of an intraluminal esophageal stent and radical resection as indicated, missed or delayed diagnosis of full-thickness esophagogastric necrosis with its prohibitive mortality was avoided and the complication of severe esophageal stricture was virtually eliminated (Group 2).(ABSTRACT TRUNCATED AT 250 WORDS)