Dearani, Joseph A.

Background: Marked septal hypertrophy is considered a requirement for effective myectomy in obstructive hypertrophic cardiomyopathy (HCM), with mitral valve replacement recommended as the alternative strategy in patients with minimal hypertrophy. However, it remains uncertain whether relief of obstruction can be effectively abolished without mitral valve replacement in patients with minimal septal wall thickening. Methods: Of 500 patients who underwent surgical myectomy from 2004 to January 2014, 21 (4.2%) were identified with a maximum LV wall thickness ≤ 15mm and constitute the study cohort. Results: All 21 patients (56 ± 10 years old; 62% male) were followed for advanced heart failure symptoms refractory to drug therapy with a maximal septal wall thickness of 13.6 ± 1.7 mm (range: 10-15 mm; ≤ 12 mm in 5 patients). Outflow obstruction ≥ 30 mmHg due to mitral valve-septal contact was present after exercise in 17 of 21 patients (range: 50-150 mmHg), and under resting conditions in 4 patients (range: 30-65 mmHg). In all patients, surgical relief of obstruction consisted of muscular resection of the basal septum with revision of abnormal and apically displaced papillary muscles, which were judged intraoperatively to be contributing to obstruction. In addition, in 10 patients (47%) myectomy alone was not sufficient to relieve obstruction due to the limited opportunity for septal reduction and adjunctive mitral valve repair was performed to shorten an elongated anterior leaflet. No patient required mitral valve replacement or incurred a ventricular septal defect. Post-operatively, 4 patients developed complete heart block requiring permanent pacemaker (1 patient with pre-operative right bundle branch block) and 1 had a cerebrovascular event. At most recent follow up 18 ± 19 months post-myectomy, septal thickness was reduced to 10 ± 2 mm, no patient had an outflow gradient at rest or with provocation, and all patients were alive with the majority asymptomatic (class I: n=13; 62% and class II n=8; 38%). Conclusion: In patients with minimal septal hypertrophy, outflow obstruction can be effectively abolished with surgical myectomy and adjunctive mitral valve repair with a small increased risk of heart block, but without the need for mitral valve replacement.

Introduction: Pulmonary valve (PV) replacement is rare, so data regarding normal hemodynamics are limited. We sought to determine the normal Doppler-derived hemodynamic profiles of various PV prostheses (PVPs) in adults. Hypothesis: Doppler-derived hemodynamic profiles of normal PVPs will vary according to prosthesis type in adult patients. Methods: We performed a retrospective review of comprehensive two dimensional and Doppler echocardiographic examinations of 327 patients (median age, 35; range 18 -83 years) with normal PVPs performed early after implantation. Data were analyzed to determine mean±SD. Results: Parasternal short axis was the most commonly used window (96%) for assessment of PVPs. For the 327 PVPs, peak instantaneous gradient was 25±11 mmHg, mean systolic gradient was 13.1±6.1 mmHg, and mean right ventricular outflow tract velocity was 0.8±0.6 m/s. Results for selected variables analyzed by prosthesis type are listed in the Table. Pericardial prostheses had significantly lower peak gradient (21±10.1 mmHg; P=0.002) compared with porcine (25±9.7 mmHg), mechanical (26±16.6 mmHg), bovine venous (34±15.5 mmHg) and homograft PVPs (35±8.4 mm Hg). Conclusions: This is the largest series reporting normal Doppler-derived hemodynamic profiles of PVPs. This study establishes normative values for Doppler-derived variables for normal PVPs implanted in adults. Pericardial prostheses had the optimal hemodynamic profile in this patient population.

Background —Patients who have had previous CABG may subsequently develop significant mitral valve (MV) dysfunction that requires surgical intervention. Methods and Results —We reviewed 80 consecutive patients who had had previous CABG and who underwent MV surgery between January 1972 and March 1997. Forty-seven (59%) had initial CABG elsewhere, and 5 had had previous CABG twice. The mean interval between the previous CABG and the MV surgery was 6.3 years (range, 4.4 months to −17 years). At least 15 patients had grade 1/4 to 2/4 mitral regurgitation at initial CABG. The study group included 59 men and 21 women, with a mean study group age of 65.5 years. Ninety-four percent had symptoms of congestive heart failure, 60% had angina, 96% were in NYHA class III to IV, and 37% had an ejection fraction (EF) <50%. Origin of the MV disease was ischemic in 33 patients, myxomatous in 19, combined ischemic and myxomatous in 16, rheumatic in 5, infective in 3, and unknown in 4. MV repair was performed in 46 patients (58%) and MV replacement (MVR) in 34. Concomitant repeat CABG was performed in 38 (48%) patients. In-hospital mortality was 7 of 80 (8.8%); no early death occurred among patients with myxomatous disease. EF <50% was the only significant predictor of early mortality. Overall 1-, 5-, and 10-year survival was 83.8%, 55.6%, and 34.4% respectively. Predictors of late cardiac death were preoperative NYHA class IV ( P =0.0006), urgent or emergency operation ( P <0.0001), use of intra-aortic balloon pump ( P =0.002), and EF <50% ( P =0.01). Seven patients had an additional reoperation: 4 received CABG, 2 MVR, and 2 MV repair. Conclusions —Ischemic, myxomatous, rheumatic, or infective MV dysfunction may develop subsequently after CABG. MVR or MV repair after previous CABG is associated with an acceptably low operative risk and good relief of symptoms. Left ventricular function is strongly correlated with both hospital and late mortality. Surgery should be done before LV dysfunction develops.

Background —Proliferation of the intima is an early lesion of saphenous vein graft disease. Early patency rates of radial artery grafts are acceptable, but little is known about their risk of intimal hyperplasia. Methods and Results —To develop a model of intimal hyperplasia, we incubated human saphenous veins, internal mammary arteries, and radial arteries (n=6, 8, and 10, respectively) in an organ culture with Rosewell Park Memorial Institute 1640 (30% serum) for 0, 4, 7, 10, and 14 days. Quantitative histological studies were performed, and the average intimal-to-medial (I/M) ratio was calculated for each incubation interval. After 10 and 14 days of culture, the I/M ratio increased in the saphenous veins ( P =0.03, P =0.04 versus 0 day, respectively). No significant increase occurred in the I/M ratio in either the internal mammary or radial arteries. Next, the ability of adenoviral gene transfers to inhibit intimal hyperplasia in the saphenous veins was evaluated. Adenoviral-mediated gene transfer of nitric oxide synthase significantly reduced the I/M ratio at 14 days compared with vehicle ( P =0.001) and virus ( P =0.004) controls. Conclusions —The human saphenous vein has a greater propensity for intimal hyperplasia than arterial grafts; the human radial artery behaves similarly to the internal mammary artery. In the future, gene therapy may augment nitric oxide synthase, limiting vein graft disease.

Objective To review the long-term functional outcomes of patients with Ebstein anomaly who had cardiac surgery at our institution. Methods From April 1, 1972 to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations at Mayo Clinic Rochester. The 506 late survivors form the basis of this report. Patient records were reviewed and all patients were mailed a medical questionnaire or contacted by telephone. Patients who had associated complex congenital heart disease were excluded (pulmonary atresia, conotruncal abnormalities, and atrioventricular discordance and ventriculoarterial discordance). Results At the initial operation at our institution, the mean age was 24 years (range 8 days to 79 years) and 53% were female. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years respectively. Surveys were returned by 285 patients. Two hundred and thirty-seven (83%) of patients were in NYHA class I or II and 34% were taking no cardiac medication. Five patients (2%) reported having endocarditis, 1 patient (<1%) reported a stroke, and 103 patients (36%) had atrial fibrillation or flutter. There were a total of 202 pregnancies among 82 women. Recurrence of congenital heart disease was low. Only 9/232 (0.04%) of liveborn children had congenital heart disease. Conclusions Patients have good long term survival and functional outcomes, after operation for Ebstein anomaly. Atrial arrhythmias are relatively common both before and after operation. Pregnancy appears to be well tolerated with low recurrence risk of congenital heart disease.

Background Coarctation of the aorta is commonly associated with recoarctation or additional cardiovascular disorders that require intervention. The best surgical approach in such patients is uncertain. Ascending-to-descending aortic bypass graft via the posterior pericardium (CoA bypass) allows simultaneous intracardiac repair or an alternative approach for the patient with complex coarctation. Methods and Results Between 1985 and 2000, 18 patients (13 males and 5 females, mean age 43±13 years) with coarctation of the aorta underwent CoA bypass through median sternotomy. Before operation, average New York Heart Association class was II (range I to IV), and 15 patients (83%) had systemic hypertension. One or more previous cardiovascular operations had been performed in 12 patients (67%); 10 patients had ≥1 prior coarctation repair. Two patients had prior noncoarctation cardiovascular surgery. Concomitant procedures performed in 14 patients (78%) included the following: aortic valve replacement in 9; coronary artery bypass surgery in 3; mitral valve repair in 2; and septal myectomy, mitral valve replacement, aortoplasty, subaortic stenosis resection, ventricular septal defect closure, and ascending aorta replacement in 1 patient each. All patients survived the operation and were alive with patent CoA bypass at a mean follow-up of 45 months. No graft-related complications occurred, and there were no instances of stroke or paraplegia. Systolic blood pressure fell from 159 mm Hg before surgery to 125 mm Hg after surgery. Conclusions CoA bypass via median sternotomy can be performed with low morbidity and mortality. Although management must be individualized, extra-anatomic CoA bypass via the posterior pericardium is an excellent single-stage approach for patients with complex coarctation or recoarctation and concomitant cardiovascular disorders.

Background —The role of surgical closure of patent foramen ovale (PFO) for cerebral infarction (CI) or transient ischemic attack (TIA) resulting from paradoxical embolism is unclear, and its effect on recurrence is unknown. Our objective was to determine the outcome of surgical closure of PFO in patients with a prior ischemic neurological event, define the rate of CI or TIA recurrence after PFO closure, and identify risk factors for these recurrences. Methods and Results —We retrospectively analyzed 91 patients (58 men, 33 women) with ≥1 previous cerebrovascular ischemic events who underwent surgical PFO closure between April 1982 and March 1998. The presence of a PFO with a right-to-left shunt was confirmed with transesophageal echocardiography. Mean age was 44.2±12.2 years. The index event was a CI in 59 and a TIA in 32; a Valsalva-like episode preceded the event in 15 patients. Deep venous thrombosis was documented in 9 patients, and a hypercoagulable state was identified in 10. Surgical closure was performed with extracorporeal circulation by either direct suture (n=82) or patch closure (n=9). Limited incisions were used in 18.7% of patients. There was no operative mortality. Morbidity included transient atrial fibrillation (n=11), pericardial drainage for effusion (n=4), exploration for bleeding (n=3), and superficial wound infection (n=1). Follow-up totaled 176.3 patient-years, and mean follow-up was 2.0 years. No one had a CI, and 8 had a TIA during follow-up, with 1 caused by temporal arteritis. Transesophageal echocardiography demonstrated all closures to be intact in these patients. The overall freedom from TIA recurrence during follow-up was 92.5±3.2% at 1 year and 83.4±6.0% at 4 years. Having multiple neurological events before PFO closure was the only significant risk factor for TIA or CI recurrence after closure by univariate analysis ( P =0.05); the small number of post-PFO closure cerebral ischemic events precluded multivariate analysis. Conclusions —Surgical closure of PFO can be performed with minimal morbidity and mortality. PFO closure may decrease the risk of recurrent stroke or TIA and may avoid lifelong anticoagulation in the young adult if there is no other indication. Recurrent cerebrovascular ischemic events after surgery should prompt further evaluation to identify causes other than paradoxical embolism.

Background Pericardiectomy for post-radiation constrictive pericarditis has been reported to generally have unfavorable outcomes. This study sought to evaluate surgical outcomes in a large cohort of patients undergoing pericardiectomy for radiation-associated pericardial constriction. Methods A retrospective analysis of all patients (≥18years) who underwent pericardiectomy for a diagnosis of constrictive pericarditis with a prior history of mediastinal irradiation from June 2002 to June 2019 was conducted. There were 100 patients (mean age 57.2±10.1 years, 49% females) who met the inclusion criteria. Records were reviewed to look at surgical approach, extent of resection, early mortality and late survival. Results The overall operative mortality was 10.1% (n=10). The rate of operative mortality decreased over the study period; however, the test of trend was not statistically significant (P=0.062). Hodgkin’s disease was the most common malignancy (64%) for which mediastinal radiation had been received. Only 27% patients had an isolated pericardiectomy, and concomitant pericardiectomy and valve surgery was performed in 46% patients. Radical resection was performed in 50% patients, whereas 47% patients underwent a subtotal resection. Prolonged ventilation (26%), atrial fibrillation (21%) and pleural effusion (16%) were the most common post-operative complications. The overall 1,5- and 10-years survival was 73.6%, 53.4% and 32.1% respectively. Increasing age (HR 1.044, 95%CI 1.017-1.073) appeared to have a significant negative effect on overall survival in the univariate model. Conclusion Pericardiectomy performed for radiation associated constrictive pericarditis has poor long-term outcomes. The early mortality, though high (~10%), has been showing a decreasing trend in the test of time.

Background: Papillary fibroelastoma (PFE) is the most common benign cardiac tumor. Because PFEs have the potential to embolize, they typically are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. Objectives: To determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence. Methods: Patients with pathologically proven PFE were retrospectively identified from a single center. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. Echocardiographic images obtained intraoperatively (after excision) and at dismissal were compared with those of the most recent examination to assess the possibility of PFE recurrence. Results: Ninety eight patients were identified. The mean (SD) duration of follow-up was 5.4 (3.7) years (range, 1-17 years). Twelve patients (12.2%) had echocardiographically proven PFE recurrence. Three patients had the recurrent lesion surgically re-excised, and pathologic analysis showed that 2 were recurrent PFEs and 1 was a Lambl excrescence. Clinical presentation of stroke or TIA was more common for the recurrence patients (for the first PFE) than for the nonrecurrence patients (83% vs 26%; P <.001). Conclusions: Contrary to published data, PFEs do recur after surgical excision. This emphasizes the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses. Table. Characteristics of Patients With Recurrent PFE Abbreviations: AV, aortic valve; LA, left atrium; LE, Lambl excrescence; LVOT, left ventricular outflow tract; MV, mitral valve; PV, pulmonary valve; RA, right atrium; RC, right cusp; TV, tricuspid valve. a Corresponds to the same location. b Corresponds to a different location .

Objective: To identify risk factors for pediatric mechanical mitral valve replacement (mMVR) to improve management in this challenging population. Methods: From 1993 to 2019, 93 children underwent 119 mMVR operations (median age, 8.8 years [interquartile range [IQR]: 2.1-13.3], 54.6% females) at our institution. Twenty-six (21.8%) patients underwent mMVR at ≤2 years and 93 (78.2%) patients underwent mMVR at >2 years. Median follow-up duration was 7.6 years [IQR: 3.2-12.4]. Results: Early mortality was 9.7%, but decreased with time and was 0% in the most recent era (13.9% from 1993 to 2000, 7.3% from 2001 to 2010, 0% from 2011 to 2019, P = .04). It was higher in patients ≤2 years compared to patients >2 years (26.9% vs 2.2%, P < .01). On multivariable analysis for mitral valve reoperation, valve size <23 mm was significant with a hazard ratio of 5.38 (4.87-19.47, P = .01);. Perioperative stroke occurred in 1% and permanent pacemaker was necessary in 12%. Freedom from mitral valve reoperation was higher in patients >2 years and those with a prosthesis ≥23 mm. Median time to reoperation was 7 years (IQR: 4.5-9.1) in patients >2 years and 3.5 years (IQR: 0.6-7.1) in patients ≤2 years ( P = .0511), but was similar between prosthesis sizes ( P = .6). During follow-up period (median 7.6 years [IQR: 3.2-12.4], stroke occurred in 10%, prosthetic valve thrombosis requiring reoperation in 4%, endocarditis in 3%, and bleeding in 1%. Conclusion: Early and late outcomes of mMVR in children are improved when performed at age >2 years and with prosthesis size ≥23 mm. These factors should be considered in the timing of mMVR.