Genetics, Pregnancy, and Aortic Degeneration.

We present a case of familial thoracic aortic aneurysm and dissection (FTAAD) in a pregnant female. FTAAD is an inherited, nonsyndromic aortopathy resulting from several genetic mutations critical to aortic wall integrity have been identified. One such mutation is the myosin heavy chain gene (MYH11) which is responsible for 1-2% of all FTAAD cases. This mutation results in aortic medial degeneration, loss of elastin, and reticulin fiber fragmentation predisposing to TAAD. Aortic disease is more aggressive during pregnancy as a result of increased wall stress from hyperdynamic cardiovascular changes and estrogen-induced aortic media degeneration. Our patient was a 29-year-old G2P1 woman at 26 weeks gestation presenting with abdominal and back pain. Work-up revealed a 6.4-cm ascending aortic aneurysm with a type A dissection extending into all arch vessels, aortic coarctation at the isthmus, and a separate focal type B aortic dissection with visceral involvement. Surgical management included concomitant cesarean section with delivery of a live premature infant, tubal ligation, ascending aortic replacement with reconstruction of the arch vessels, and aortic valve resuspension. The type B dissection was managed medically without complication. This is the first reported case of aortic dissection in a patient with FTAAD/MYH11 mutation and pregnancy. This case highlights that FTAAD and pregnancy cause aortic degeneration via distinct mechanisms and that hyperdynamics of pregnancy increase aortic wall stress. Management of pregnancy associated with aortopathy requires early transfer to a tertiary center, careful investigation to identify familial aortopathy, fetal monitoring, and a multidisciplinary team approach.

Combined heart-kidney transplant improves post-transplant survival compared with isolated heart transplant in recipients with reduced glomerular filtration rate: Analysis of 593 combined heart-kidney transplants from the United Network Organ Sharing Database.

OBJECTIVE: Criteria for simultaneous heart-kidney transplant (HKTx) recipients are unclear. We characterized the evolution of combined HKTx in the United States over time compared with isolated heart transplantation (HTx) and determined factors maximizing post-transplant survival. We focused on whether a threshold estimated glomerular filtration rate (eGFR) could be identified that justified combined transplantation. METHODS: A supplemented United Network Organ Sharing Dataset identified HTx and HKTx recipients from 2000 to 2010. eGFR was calculated for HTx and recipients were grouped into eGFR quintiles. Time-related mortality was compared among recipients, with multivariable factors sought using Cox proportional hazard regression models. RESULTS: We identified 26,183 HTx recipients, of whom 593 were HKTx recipients. HTx increased modestly over time (3.6%), whereas prevalence of HKTx increased dramatically (147%). Risk-unadjusted survival was similar among HTx recipients (8.4 ± 0.04 years) and HKTx recipients (7.7 ± 0.2 years) (P = .76). Isolated HTx recipients in the lowest eGFR quintile had decreased survival (P < .001), but those in the third eGFR quintile had superior survival, suggesting a benefit in this subgroup. HTx recipients in the lowest eGFR quintile (eGFR less than mean 37 mL/minute) had worse survival than combined HKTx recipients (7.1 ± 0.07 vs 7.7 ± 0.2; P < .001). Multivariable factors for increased mortality among HTx recipients included lower eGFR, higher recent panel reactive antibody score, older age, African American race, diabetes, longer ischemic time, and certain diagnoses. CONCLUSIONS: Performance of combined HKTx is increasing out of proportion to isolated HTx. eGFR is an important determinant of improved HTx survival. Combined HKTx recovers post-transplant survival in patients with eGFR <37 mL/minute and can be recommended in this subgroup.

Safety and efficacy of prothrombin complex concentrates for the treatment of coagulopathy after cardiac surgery.

OBJECTIVE: Coagulopathy is an important cause of bleeding after complex cardiac surgery. The conventional treatment for coagulopathy is transfusion, which is associated with adverse outcomes. We report our initial experience with the prothrombin complex concentrate FEIBA (factor VIII inhibitor bypassing activity) for the rescue treatment of coagulopathy and life-threatening bleeding after cardiac surgery. METHODS: Twenty-five patients who underwent cardiac surgery with coagulopathy and life-threatening bleeding refractory to conventional treatment received FEIBA as rescue therapy at our institution. This cohort represents approximately 2% of patients undergoing cardiac surgery in our university-based practice during the study. RESULTS: The patients were at high risk for postoperative coagulopathy with nearly all patients having at least 2 risk factors for this. Aortic root replacement (Bentall or valve-sparing procedure) and heart transplant with or without left ventricular assist device explant were the most common procedures. The mean FEIBA dose was 2154 units. The need for fresh frozen plasma and platelet transfusion decreased significantly after FEIBA administration (P = .0001 and P < .0001). The mean internationalized normalized ratio decreased from 1.58 to 1.13 (P < .0001). Clinical outcomes were excellent. No patient returned to the operating room for reexploration. There was no hospital mortality and all patients were discharged home. One patient who had a central line and transvenous pacemaker developed an upper extremity deep vein thrombosis. CONCLUSIONS: Our initial experience with FEIBA administration for the rescue treatment of postoperative coagulopathy and life-threatening bleeding has been favorable. Further studies are indicated to confirm its efficacy and safety and determine specific clinical indications for its use in patients undergoing cardiac surgery.

Right ventricular outflow tract cannulation for right ventricular assist device implantation.

A need persists for implantable devices that provide support for the failing right ventricle. The anatomy of the right ventricle presents unique challenges at the time of right ventricular assist device implant. We describe a technique for right ventricular outflow tract cannulation that minimizes the risk of right ventricular assist device inflow cannula obstruction and right ventricular compression.

Mechanical circulatory support pathways that maximize post-heart transplant survival.

BACKGROUND: Heart transplant (HTx) recipients reach transplantation through increasing numbers of support pathways, including transition from one pathway to another. Outcomes of patients successfully bridged with various support pathways are unknown. We sought to identify mechanical circulatory support pathways that maximize survival after HTx. METHODS: A supplemented United Network Organ Sharing Dataset tracked status 1 HTx outcomes from 2000 to 2010. Recipients were grouped based on support pathway before HTx, including those transitioning from one pathway to another. Multivariable factors for time-related death were sought using Cox proportional hazard regression models. RESULTS: We identified 13,250 status 1 HTx recipients. Initial support pathways were inotropes (n = 7,607), left ventricular assist device (LVAD [n = 4,034]), intraaortic balloon pump (n = 729), biventricular assist device (n = 521), extracorporeal membrane oxygenation (ECMO [n = 316]), and right ventricular assist device (n = 43). Multivariable analysis demonstrated that LVAD use conferred a survival advantage (hazard ratio [HR] 0.71; p < 0.001), whereas all other support pathways, including inotropes (HR 1.1; p = 0.02), right ventricular assist device (HR 1.9; p = 0.01), and ECMO (HR 2.2; p < 0.001) increased the risk of post-HTx death. Support pathway transition (both escalation and reduction) occurred in 2,175 patients. Patients who transitioned from either ECMO or biventricular assist device support at listing to LVAD-only support at HTx had improved post-HTx survival that was comparable to patients who had LVAD-only therapy throughout their course (p = 0.74). CONCLUSIONS: The LVAD supported HTx recipients have better posttransplant survival than patients after all other mechanical support pathways. Survival after HTx is optimized when ECMO or biventricular assist device support can be transitioned to LVAD-only support. Our findings should aid clinical decision making and inform organ allocation policy development intended to maximize societal benefits of HTx.

Left atrial plication for heart transplantation in patients with giant left atrium.

Giant left atrium occasionally occurs in patients undergoing heart transplantation and causes a technical challenge for the surgeon because of the substantial discrepancy in size between the left atrial cuffs of the recipient and donor. We describe a left atrial plication technique that substantially reduces this discrepancy and allows for a standard left atrial anastomosis to be performed without any other modifications in technique.

Impact of single-ventricle physiology on death after heart transplantation in adults with congenital heart disease.

BACKGROUND: Prevalence of univentricular (1V) anatomy over time and whether 1V anatomy is associated with early death after heart transplant (HTx) among recipients with adult congenital heart disease (ACHD) is unknown. We investigated changes in case-mix over time, 1V vs biventricular (2V) status, and the effect of 1V anatomy on death after HTx among ACHD recipients. METHODS: The Nationwide Inpatient Sample (NIS) was used to identify ACHD HTx recipients in the United States aged 14 years or older from 1993 to 2007, divided into era 1 (1993 to 2000) and era 2 (2001 to 2007). In-hospital death was compared among recipients with 1V and 2V anatomy. Multivariable determinants associated with an increased risk of in-hospital death were sought with logistic regression models. RESULTS: From a national estimate of 509 ACHD recipients, 143 were 1V and 366 were 2V. Overall, 1V in-hospital mortality (23%) was higher than for 2V (8%; p<0.001) and remained associated with in-hospital death after adjustment for other factors (odds ratio, 3.9; 95% confidence interval, 1.29 to 11.74; p=0.02). All 1V diagnoses had higher mortality than all 2V diagnoses. Despite minor fluctuations, the proportion of 1V patients did not increase over time (era 1, 36%; era 2, 30%; p=0.46). CONCLUSIONS: Overall case-mix of ACHD recipients (1V vs 2V) has not changed over time. Initial 1V anatomy increases post-HTx death among ACHD recipients, whereas 2V patients have mortality rates similar to non-CHD recipients. National and international transplant registries should include specific CHD diagnoses because this factor plays such a large role in determining early outcomes.

Patient-specific insulin-resistance-guided infusion improves glycemic control in cardiac surgery.

BACKGROUND: Maintenance of appropriate capillary blood glucose in cardiovascular surgery patients has been associated with improved outcomes, including lower mortality. Interpatient variability in insulin resistance can make management difficult, leading to unexpected episodes of hypoglycemia and hyperglycemia. To improve postoperative glucose control at our institution, a patient-specific insulin-resistance-guided (IRG) protocol was developed. METHODS: Prospective data were gathered on 100 consecutive cardiovascular surgery patients managed with our standard insulin infusion protocol and 100 patients managed using the IRG protocol. Clinical characteristics and glycemic indices were analyzed for the two groups. Primary endpoints included (1) percentage of time spent in the target range, (2) number of hypoglycemic and hyperglycemic episodes, (3) time to achievement of target blood glucose, and (4) the total daily dose of insulin required. RESULTS: The IRG protocol resulted in significant improvements, including increased percentage of time spent in the normoglycemic range (82.5% versus 65.8%, p < 0.001), reduced rate of hypoglycemic episodes (0.12 versus 0.99, p < 0.01), reduced rate of hyperglycemic episodes (capillary blood glucose >126 mg/dL: 4.8 versus 8.2, p < 0.01), and a reduced time to the first measurement in the target range. Total daily dose of insulin was mildly increased, but failed to reach statistical significance (92.48 versus 82.64 units, p = 0.32). CONCLUSIONS: Use of the IRG protocol led to improved glycemic indices while reducing episodes of hypoglycemia in both diabetic and nondiabetic patients. The ability to adjust a patient's insulin dosing based upon factors related to their insulin resistance results in improved blood glucose control and safety in cardiovascular surgery patients.

Favorable early outcomes for patients with extended indications for thoracic endografting.

BACKGROUND: Endografts originally designed and approved for the treatment of thoracic aortic aneurysms have rapidly been adopted for nonapproved use in the treatment of disorders of the thoracic aorta, including aortic transection, dissection, pseudoaneurysms, and thoracoabdominal aneurysms. The purpose of this study was to evaluate the early outcomes of patients treated with thoracic endografts for nonapproved indications at our institution. METHODS: The medical records of patients undergoing thoracic endografting at our institution from August 2005 until March 2008 were reviewed. Patients undergoing endografting for uncomplicated thoracic aortic aneurysms were excluded. The outcomes of patients with extended indications for thoracic endografting were studied. RESULTS: During the study period, endografting was performed in 31 patients for nonapproved aortic conditions. Patients underwent endografting for a spectrum of indications, including aortic transection (n = 12), complications of type B aortic dissection including rupture (n = 9), thoracoabdominal aneurysm with visceral debranching (n = 6), aortic arch debranching (n = 2), and pseudoaneurysm associated with prior coarctation repair (n = 2). Early outcomes were favorable. All patients had successful endograft repair of their anatomic lesion. There were no endoleaks. There was no hospital mortality. Average hospitalization was 15 days for patients with aortic transection and 9 days for all other patients. CONCLUSIONS: Thoracic endografts are versatile devices that with appropriate expertise can be used effectively to treat a spectrum of disorders of the thoracic aorta, including acute emergencies. Early outcomes of patients with extended indications for thoracic endografting compare favorably to published series of patients treated with open procedures. Further study is required to assess the long-term efficacy of these devices.

Improved quality and cost-effectiveness of coronary artery bypass grafting in the United States from 1988 to 2005.

OBJECTIVE: This study was undertaken to assess the impact of increasing patient complexity and health care cost on coronary artery bypass grafting quality and cost-effectiveness in the United States over an 18-year period. METHODS: A retrospective study was carried out utilizing the Nationwide Inpatient Sample to track the characteristics and outcomes of 5,549,700 patients having isolated coronary artery bypass grafting in the United States from 1988 to 2005. Expected mortality, risk-adjusted mortality, and hospital charges were tracked over this period. RESULTS: The prevalence of congestive heart failure, pulmonary disease, diabetes, and acute myocardial infarction increased significantly over the study period. Expected mortality increased from 2.57% to 3.66%, reflecting the increasing patient comorbidity burden (P < .0001). Despite this, coronary artery bypass grafting outcomes improved, leading to a decrease in risk-adjusted mortality from 6.20% to 2.12% (P < .0001). Furthermore, when hospital charges were corrected for medical care inflation, hospital charges declined significantly, from $26,210 in 1988 to $19,196 in 2005 (1988 dollars, P < .0001). CONCLUSIONS: Coronary artery bypass grafting surgery is being performed on an increasingly complex, high-risk patient population in the United States. Despite this challenge, risk-adjusted operative mortality has progressively declined. Moreover, hospital charges for coronary artery bypass grafting in relation to other medical care services have been reduced. These findings reflect improved quality and cost-effectiveness of coronary artery bypass grafting in the United States. Ongoing efforts directed at quality improvement should address the risks associated with comorbidities that increasingly accompany the diagnosis of coronary artery disease in patients having coronary artery bypass grafting.

Traumatic thoracic aortic transection in small-diameter aortas: percutaneous endograft repair.

The authors describe percutaneous repair of traumatic aortic transection in small-caliber aortas by using iliac extender stent-grafts in three patients (two female and one male patient; average age, 29.3 years). The average aortic diameters were 18.7 mm proximal and 16.4 mm distal to the tear. Iliac limb extenders (55 mm in length, 20-22 mm in diameter) were transferred from their 55-cm long delivery sheaths into 80-cm 16- or 18-F sheaths. The long sheaths enabled the percutaneous delivery of multiple stent-grafts to the thoracic aorta via a single femoral sheath. The transections were successfully treated in all patients without complication.

Treatment of endocarditis: a decade of experience.

BACKGROUND: Endocarditis represents a small proportion of cardiovascular disease but is associated with high mortality. Previous studies have reported a range of outcomes, and determinants of mortality remain poorly defined. METHODS: The goal of this retrospective study was to identify independent variables for early and late mortality in 364 consecutive patients with endocarditis over a 10-year period. RESULTS: The mean age of patients was 48.2 years, 35% had a history intravenous drug use, 19.8% were reoperative, and 93% had native valve endocarditis. Fever (68%) and fatigue (36%) were the most common presenting symptoms, and congestive heart failure (52%), embolization (45%), and uncontrolled sepsis (36%) were the most common indications for surgery. Overall survival at discharge, 1, 5, and 10 years was 87%, 76%, 55%, and 31%, respectively. Survival at discharge, 5, and 10 years was 91%, 69%, and 41% for surgical patients and 85%, 60%, and 31% for medically treated patients, respectively. Surgery was associated with improved short-term and long-term survival (p < 0.0.01). Independent predictors of early death were hemodynamic instability (p = 0.013) and age older than 55 years (p < 0.025). Medical treatment (p = 0.005), age older than 55 years (p = 0.032), institution (p < 0.001), New York Heart Association functional class III or IV (p = 0.002), and hemodynamic instability (p = 0.044) were predictive of late death. CONCLUSIONS: Short-term and long-term mortality from endocarditis remains high, although surgically treated patients had improved survival. Differing outcomes from two geographically similar institutions highlight the limitations of extrapolating risk factors between disparate patient populations.

Becker's muscular dystrophy and orthotopic heart transplantation: perioperative considerations.

Patients with Becker's and Duchenne's muscular dystrophy occasionally have myocardial involvement leading to end-stage heart failure. Heart transplantation is established as an effective therapy. Achieving successful outcomes in this challenging group requires special consideration during the perioperative period to limit preoperative deconditioning, minimize anesthesia complications, and rapidly institute rehabilitation with appropriate precautions. We reviewed our recent experience with Becker's muscular dystrophy patients and discuss the management of perioperative issues specific to this patient group.

Spontaneous rupture of the thoracic aorta.

Spontaneous rupture of the aorta without previous history of trauma, hypertension, or apparent aortic pathology is exceedingly rare. Delayed or nonoperative repair of this condition is usually lethal. Survival after spontaneous mid arch aortic rupture requires a high index of suspicion, rapid and appropriate diagnostic tests, and early operative repair. Clinical presentation, clues to diagnosis, and optimal management of this entity are discussed.

Leiomyomatosis metastatic to the tricuspid valve complicated by pelvic hemorrhage.

Metastatic leiomyomatosis is a rare but potentially life-threatening tumor of smooth muscle cells. Leiomyomas originate predominantly in the uterus and can spread to the lung and, rarely, the heart. We present a case of a 36-year-old woman with known pelvic leiomyomatosis with metastasis to the tricuspid valve. Tricuspid valve replacement was complicated by retroperitoneal hemorrhage from residual pelvic tumor. This potentially catastrophic occurrence should be considered when undertaking resection of intracardiac leiomyomatosis.

Crush injury and rhabdomyolysis.

Crush injuries resulting in traumatic rhabdomyolysis are an important cause of acute renal failure. Ischemia reperfusion is the main mechanism of muscle injury. Intravascular volume depletion and renal hypoperfusion, combined with myoglobinuria, result in renal dysfunction. The infusion of intravenous fluids before extrication or soon after injury may lessen the severity of the crush syndrome. Serum CK levels can be used to screen patients with crush injuries to determine injury severity. Once intravascular volume has been stabilized, and the presence of urine flow has been confirmed, a forced mannitol-alkaline diuresis for prophylaxis against hyperkalemia and acute renal failure should be instituted. If an extremity compartment syndrome is suspected, one should have a low threshold for checking the intracompartmental pressures. Further studies are needed to demonstrate if any treatment regimen is truly superior to early, aggressive crystalloid infusion.