Spontaneous intracranial hypotension in patients with bicuspid aortic valve.

BACKGROUND AND AIM OF THE STUDY: Spontaneous intracranial hypotension, an important but frequently misdiagnosed cause of headache, is often associated with the presence of an underlying systemic connective tissue disorder. Bicuspid aortic valve (BAV) is a relatively common systemic connective tissue disorder that has been associated with other causes of headache, such as intracranial aneurysm and cervico-cephalic arterial dissection. The study aim was to assess the frequency of BAV among patients with spontaneous intracranial hypotension. METHODS: The medical records of a consecutive group of patients with spontaneous intracranial hypotension were reviewed for the presence of BAV. The control population consisted of a group of patients evaluated for face pain or headache not due to spontaneous intracranial hypotension. RESULTS: The presence of a BAV was confirmed in three of 273 patients (1.1%) with spontaneous intracranial hypotension, but in none of 506 controls (p = 0.04). CONCLUSION: Patients with BAV may be at an increased risk of spontaneous intracranial hypotension. The aortic valvular cusps, as well as the spinal dura, are derived from cells that originate in the neural crest; hence, a disorder of neural crest cell migration could explain the findings of BAV and spontaneous intracranial hypotension in the presently reported patients.

Screening for intracranial aneurysms in patients with bicuspid aortic valve.

OBJECTIVE: Bicuspid aortic valve (BAV) is a common congenital heart defect affecting half to 2% of the population. A generalized connective tissue disorder also involving the intracranial arteries has been suspected in this patient population. We therefore screened a group of patients with BAV for the presence of intracranial aneurysms. METHODS: Magnetic resonance angiography or CT angiography of the brain was used in 61 patients with BAV (age, 29-70 years [mean 48 years]) and in 291 controls (28-78 years [mean 56 years]). RESULTS: Intracranial aneurysms were detected in 6 of 61 patients with BAV (9.8%; 95% confidence interval [CI] 2.4%-17.3%). This was significantly higher than in the control population (3/291 [1.1%; 95% CI 0%-2.2%]) (p = 0.0012). Female sex (p = 0.02) and advanced age (p = 0.003), risk factors for intracranial aneurysm development, were more common in the control population than among the patients with a BAV. No significant differences were detected in age, sex, smoking, arterial hypertension, alcohol use, aortic diameter, or frequency of aortic coarctation between BAV patients with and without intracranial aneurysms. CONCLUSION: In this case-control study, the frequency of intracranial aneurysms among our bicuspid aortic valve patient population was significantly higher than in the control population.

Coronary revascularization alone or with mitral valve repair: outcomes in patients with moderate ischemic mitral regurgitation.

We sought to evaluate retrospectively the outcomes of patients at our hospital who had moderate ischemic mitral regurgitation and who underwent coronary artery bypass grafting (CABG) alone or with concomitant mitral valve repair (CABG+MVr).A total of 83 patients had a reduced left ventricular ejection fraction and moderate mitral regurgitation: 28 patients underwent CABG+MVr, and 55 underwent CABG alone. Changes in mitral regurgitation, functional class, and left ventricular ejection fraction were compared in both groups.The mean follow-up was 5.1 +/- 3.6 years (range, 0.1-15.1 yr). Reduction of 2 mitral-regurgitation grades was found in 85% of CABG+MVr patients versus 14% of CABG-only patients (P < 0.0001) at 1 year, and in 56% versus 14% at 5 years, respectively (P = 0.1), as well as improvements in left ventricular ejection fraction and functional class. One- and 5-year survival rates were similar in the CABG+MVr and CABG-only groups: 96% +/- 3% versus 96% +/- 4%, and 87% +/- 5% versus 81% +/- 8%, respectively (P = NS). Propensity analysis showed similar results. Recurrent (3+ or 4+) mitral regurgitation was found in 22% and 47% at late follow-up, respectively.In patients with moderate ischemic mitral regurgitation, either surgical approach led to an improvement in functional class. Early and intermediate-term mortality rates were low with either CABG or CABG+MVr. However, an increased rate of late recurrent mitral regurgitation in the CABG+MVr group was observed.

Use of cardiac allografts with mild and moderate left ventricular hypertrophy can be safely used in heart transplantation to expand the donor pool.

OBJECTIVES: The purpose of this study was to evaluate outcomes of heart transplantation (HTx) and changes in left ventricular wall thickness (LVWT) post-HTx using donors with left ventricular hypertrophy (LVH). BACKGROUND: Limited data are available on use of donor hearts with LVH in HTx. METHODS: We reviewed 427 patients who underwent HTx: 62 received hearts with LVH (interventricular septum [IVS] or posterior wall [PW] thickness >or=1.2 cm) by echocardiography, and 365 received hearts without LVH. The median follow-up was 3.8 years (range 0 to 16.2 years). RESULTS: Recipient age was 56 +/- 11 years and donor age was 30 +/- 12 years. Baseline recipient characteristics were similar in both groups. Donors with LVH were older (35 +/- 12 years vs. 29 +/- 12 years, p = 0.001) and had higher rates of intracranial hemorrhage (38% vs. 15%, p = 0.001). The LVWT was increased in the LVH group compared with LVWT in the non-LVH group (IVS: 1.28 +/- 0.18 cm vs. 0.85 +/- 0.19 cm, PW: 1.27 +/- 0.19 cm vs. 0.85 +/- 0.20 cm, p = 0.0001 for both groups). Mild LVH (1.2 to 1.3 cm) was found in 42%, moderate (>1.3 to 1.7 cm) in 53%, and severe (>1.7 cm) in 5% of donors with LVH. Left ventricular wall thickness regression occurred in both IVS and PW (1.28 +/- 0.18 cm vs. 1.10 +/- 0.13 cm vs. 1.13 +/- 0.14 cm, and 1.27 +/- 0.19 cm vs. 1.11 +/- 0.11 cm vs. 1.13 +/- 0.14 cm, at baseline, 1 year, and 5 years, respectively; p < 0.001 for change from baseline to 1 and 5 years for both locations). Patients with or without donor LVH had similar 1-year (3.5% vs. 9.5%, p = 0.2) and 5-year survival rates (84 +/- 5.9% vs. 70 +/- 2.7%, p = 0.07). CONCLUSIONS: Short- and long-term survival rates and rates of LVH at follow-up were similar in both groups, suggesting that donor hearts with mild and moderate LVH can be safely used in HTx.

Pre-existing pulmonary hypertension in patients with end-stage heart failure: impact on clinical outcome and hemodynamic follow-up after orthotopic heart transplantation.

BACKGROUND: The purpose of this investigation was to determine the influence of pre-existing pulmonary hypertension (PHT) on outcome and to assess the pulmonary hemodynamic changes after heart transplantation (HT). METHODS: A total of 410 patients were studied before and after (1 month and 1 year) HT: Group 1 (n = 266) had no PHT (PVR < 3 Wood units [WU], TPG < 10 mm Hg); Group 2 (n = 112) had mild-moderate PHT (PVR 3 to 6 WU, TPG 10 to 20 mm Hg); and Group 3 (n = 32) had severe PHT (PVR > 6 WU, TPG > 20 mm Hg). RESULTS: Mean (+/- SD) follow-up was 5.2 +/- 4.1 years, mean recipient age was 57 +/- 11, and mean donor age was 30 +/- 12 years. Baseline characteristics were similar in all groups, except donor/recipient weight ratio, which was higher in patients with PHT (p = 0.002). There was a significant (p < 0.0001) decrease in mean TPG to 11.0 within the first month and to 9.5 mm Hg after the first year. Decreases in PVR to 2.2 and 2.0 WU at 1 month and 1 year, respectively (p < 0.0001 for both) were also found. Reversibility (after vasodilation) of PHT was obtained in 85% of patients in Group 2 and in 84% in Group 3. Patients' PHT did not show a significant difference in 30-day mortality (p = 0.9) and long-term survival (p = 0.8). Patients with residual post-transplant PHT (PVR > or = 3 WU) had reduced long-term survival (p = 0.03). Multivariate analysis showed no evidence that elevated PVR was associated with death. CONCLUSIONS: Pre-existing elevated PVR that responds to vasodilator challenge does not have a negative influence on short- and long-term survival after HT. We found that residual post-transplant PHT is associated with decreased long-term survival.

Cavo-atrial tumor resection under total circulatory arrest without a sternotomy.

Surgical management of intracardiac tumors arising in the inferior vena cava often requires total circulatory arrest for safe and adequate resection. Total circulatory arrest has traditionally been accomplished by accessing the great vessels through a sternotomy. Combination of a sternotomy and a large abdominal incision results in excellent exposure but also creates the potential for significant morbidity. We report here the resection of cavoatrial tumors by achieving total circulatory arrest through femoral arterial and venous cannulation without requiring a sternotomy. This minimal-access total circulatory approach has the potential to greatly diminish morbidity when managing tumors of the inferior vena cava.

In the current era, complete revascularization improves survival after coronary artery bypass surgery.

BACKGROUND: Complete revascularization has been the standard for coronary bypass grafting. However, surgical intervention has evolved with increasing use of arterial conduits and off-pump techniques. METHODS: Patients undergoing non-redo bypass surgery from January 1998 through December 2000 were followed up with questionnaires and telephone contact. Incomplete revascularization was defined as absence of bypass grafts placed to a coronary territory supplied by a vessel with 50% or greater stenosis. RESULTS: One thousand thirty-four patients were followed for a mean of 3.3 +/- 1.6 years. Complete revascularization was found in 937 (90.6%) patients, and incomplete revascularization was found in 97 (9.4%) patients. Eight hundred twenty-seven (80.4%) patients underwent on-pump operations, and 207 (19.6%) underwent off-pump operations. Incomplete revascularization was more prevalent in off-pump versus on-pump operations (21.7% vs 6.3%, P < .001). Multivariable Cox regression analysis indicated that in-hospital cerebrovascular accidents (hazard ratio, 5.49; P < .001), chronic obstructive pulmonary disease (hazard ratio, 1.97; P = .019), and incomplete revascularization (hazard ratio, 1.85; P = .040) predicted an increased hazard (risk) of cardiac death. Left internal thoracic artery (hazard ratio, 0.38; P = .047), right internal thoracic artery (hazard ratio, 0.25; P = .019), and radial artery (hazard ratio, 0.36; P < .001) grafting reduced the risk of cardiac death. The 5-year unadjusted survival rate was 52.6% versus 82.4% in patients undergoing incomplete and complete revascularization ( P < .001), with cardiac survival rates of 74.5% versus 93.1%, respectively ( P < .001). However, this difference in cardiac survival was smaller in octogenarians with incomplete versus complete revascularizations (77.4% vs 87.6%, P = .101) and was essentially absent in off-pump versus on-pump operations if complete revascularization was achieved in both cases (93.6% vs 93.1%, P > .200). CONCLUSIONS: Complete revascularization and arterial grafting improve 5-year survival. Off-pump techniques do not affect survival. Complete revascularization should be performed whenever possible.

Ischemic mitral regurgitation: revascularization alone versus revascularization and mitral valve repair.

BACKGROUND: In this study we compared the surgical management of ischemic mitral regurgitation (IMR) by revascularization alone and by revascularization combined with mitral valve repair. METHODS: We studied 355 patients who underwent revascularization alone (n = 168) or revascularization combined with mitral valve repair (n = 187) for IMR from March 1994 to September 2003. Preoperative and operative characteristics, postoperative mitral regurgitation severity, operative mortality, and late survival were examined for each surgical group. RESULTS: No differences were noted between the two groups in age, sex, history of diabetes or hypertension, and number of bypass grafts. The combined surgical group had a lower preoperative left ventricular ejection fraction (0.38 +/- 0.14 versus 0.44 +/- 0.15), greater severity of IMR, higher frequency of prior myocardial infarction, and longer cross-clamp and pump times (p < 0.01). The combined surgical group had a greater reduction in IMR grade (2.7 +/- 0.1 grades versus 0.2 +/- 0.1 grade), a lower postoperative IMR grade (0.9 +/- 0.1 versus 2.3 +/- 0.1), and a higher success with reduction of IMR by two or more grades (89% versus 11%) (p < 0.001). In patients with 3+ or 4+ IMR, both groups had similar operative mortality (11.0% in the combined group compared with 4.7% for revascularization alone, p = 0.11) and actuarial survival at 5 years (44% +/- 5% versus 41% +/- 7%, p = 0.53). Independently predictive of higher early mortality (< or = 30 days) by Cox analysis were longer pump time (p < 0.001) and older age (p < 0.02). Predictive of late mortality (> 30 days) were older age (p < 0.001), fewer bypass grafts (p < 0.01), and lower ejection fraction (p < 0.01). After adjustment for these variables, there was a trend (p = 0.08) toward a higher late survival with the combined surgical procedure. CONCLUSIONS: In patients with IMR, combined mitral valve repair and revascularization resulted in less postoperative mitral regurgitation and similar 5-year survival when compared with revascularization alone. Attempts to reduce pump time by using off-pump techniques may reduce early mortality in these high-risk patients.

Total circulatory arrest for the replacement of the descending and thoracoabdominal aorta.

HYPOTHESIS: Hypothermic total circulatory arrest (TCA) in the resection and replacement of the thoracoabdominal and descending thoracic aorta is safe, will significantly decrease the incidence of postoperative renal failure, and should be preferentially performed over left heart bypass (LHB). DESIGN: Retrospective review case series. SETTING: Large, private, urban teaching hospital. PATIENTS: All adult patients with aortic disease that involved the distal aortic arch, the descending thoracic aorta, or the thoracoabdominal aorta who underwent resection and graft replacement of the diseased segment via LHB or TCA at our institution from 1989 to 2001 are included in this study. A total of 59 patients were evaluated: 10 had descending thoracic aneurysms, 20 had thoracoabdominal aneurysms, 22 had chronic type B dissections, 4 had acute type B dissections, and 3 had adult coarctations. INTERVENTIONS: In 1989 to 1994, LHB was primarily used; in 1994 to 2001, TCA was primarily used. MAIN OUTCOME MEASURES: Renal failure, 30-day operative mortality, paraplegia, and any other morbidities. RESULTS: A significant decrease occurred in the incidence of postoperative renal failure from 15% (3/20) in patients who underwent LHB to 0% (0/39) in patients who underwent TCA (P = .04). Furthermore, a significant decrease occurred in the 30-day operative mortality, which decreased from 20% (4/20) in patients who underwent LHB to 5% (2/39) in patients who underwent TCA (P = .04). Postoperative paraplegia decreased from 5% (1/20) in patients who underwent LHB to 2.6% (1/39) in patients who underwent TCA (P > .99). CONCLUSIONS: Our use of TCA in the resection and replacement of the diseased thoracoabdominal and descending thoracic aorta has produced excellent results. Our patients have experienced no postoperative renal failure and a low 30-day operative mortality. The use of TCA in this patient population is a viable option for surgeons comfortable with the technique.

Biventricular support in patients with profound cardiogenic shock: a single center experience.

Options for managing heart failure patients with cardiogenic shock refractory to inotropic and intra-aortic balloon pump (IABP) therapy are limited. Ventricular assist devices (VADs) can bridge these patients to heart transplantation. However, controversy exists over whether extracorporeal membrane oxygenation (ECMO) before VAD placement is beneficial. We report our use of biventricular assist devices (BiVADs) as a direct bridge to transplant. Since July 1999, 19 Thoratec BiVADs were implanted for heart failure unresponsive to medical therapy. Patient ages ranged from 20 to 67 years. Causes of heart failure included idiopathic 32%, ischemic 26%, postcardiotomy 21%, and other 21%. All patients were in cardiogenic shock, and three were receiving cardiopulmonary resuscitation (CPR) before implant. Preimplant conditions included IABP 89%, mechanical ventilation 68%, three or more inotropes 84%, hyperbilirubinemia 59%, acute renal failure 63%, and hemodialysis 16%. Fifty-nine percent of patients bridged successfully to transplantation, with 90% posttransplant survival. Duration of BiVAD support ranged from 0 to 91 days, with two patients currently on support awaiting transplantation. Complications included bleeding requiring reoperation 26%, stroke 11%, infection (any positive culture) 68%, and cannula site infection 5%. The Thoratec BiVAD can successfully be used as a direct bridge to transplantation in heart failure patients with cardiogenic shock.

Transesophageal echocardiographic evaluation of perioperative systolic murmur in aortic pathology.

Development of a new systolic murmur in patients following a Bental procedure with a prosthetic or homograft aortic valve usually indicates an aortic valve-related complication. Here, we report new etiologies of a loud systolic murmur in patients with aortic disease. One patient developed a new loud systolic murmur as an initial manifestation of acute type A aortic dissection without any complication, and two patients developed a loud systolic murmur as the major manifestation of aortic graft failure following aortic root surgery. Auscultation of a new loud systolic murmur in the upper chest in patients with known aortic disease should alert one to a complication within the ascending aorta.

Off-pump coronary surgery: effect on early mortality and stroke.

BACKGROUND: Avoiding cardiopulmonary bypass in coronary artery bypass grafting is thought to reduce early mortality and morbidity. METHODS: We used our prospective database to compare all patients having off-pump coronary surgery (n = 389) with those having on-pump coronary surgery (n = 2412) between March 15, 1995, and November 1, 2000. Patients were grouped by age (years) in decades (>90, 80-89, 70-79, 60-69, <60 years). The Northern New England risk model was applied. Thirty-two independent variables were entered into a stepwise logistic regression analysis with the end points being surgical mortality and postoperative stroke. RESULTS: Patients undergoing off-pump operations were older (70.9 +/- 12 vs 68.1 +/- 11 years; P <.001), and their Northern New England predicted risk was higher (11.9% +/- 13% vs 9.2% +/- 10%; P <.001). However, patients having on-pump bypass had significantly more bypass grafts constructed (3.3 +/- 0.8 vs 1.9 +/- 0.8; P <.001) and triple-vessel coronary artery disease (58% vs 28%; P <.001). There were no significant differences in postoperative mortality, stroke rate, complications, and length of stay between the groups. Logistic regression analysis did not show that cardiopulmonary bypass was a risk factor for either surgical mortality (odds ratio, 1.08; P =.83) or stroke (odds ratio, 1.59; P =.27). CONCLUSION: Off-pump coronary bypass did not reduce early mortality and morbidity. Early and late results should be compared in a prospective randomized study.