Sameh M. Said

Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction.

BACKGROUND Reproducible repair of Ebsteins malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction. METHODS Between June 2007 and December 2011, 89 patients (47 female; 53%) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48%), cyanosis in 29 (33%), and heart failure in 13 (15%). Prior tricuspid valve repair was performed in 12 patients (13%). Severe tricuspid regurgitation (TR) was present in 75 patients (84%). RESULTS All patients underwent cone reconstruction (360-degree leaflet tissue repair anchored at true annulus). Modifications included ringed annuloplasty in 57 patients (64%), leaflet augmentation in 28 patients (31%), and autologous chordae in 17 patients (19%). Bidirectional cavopulmonary anastomosis was performed in 21 patients (24%). Early mortality occurred in 1 patient (1%). Early reoperation for recurrent TR occurred in 12 patients (13%); re-repair was performed in 6 patients (50%), and 6 (50%) required replacement. Mean follow-up was 19.7 ± 24.7 months. There was no late mortality or reoperation. At follow-up, 72 patients (87%) had no or mild TR, 9 (11%) had moderate TR, and 2 patients (2%) had severe TR. Ringed annuloplasty was associated with less than moderate TR at dismissal (p = 0.01). CONCLUSIONS The learning curve for cone reconstruction is steep, but early mortality is low. Cone reconstruction with ringed annuloplasty results in less TR and should be used whenever possible. Longer follow-up is essential to determine late durability of cone reconstruction.

Strategies for Tricuspid Re-Repair in Ebstein Malformation Using the Cone Technique

BACKGROUND Successful tricuspid repair for Ebstein malformation (EM) is challenging. Cone repair has recently emerged as the most anatomic repair technique. There are no data on tricuspid re-repair in EM. We reviewed our experience with tricuspid valve (TV) re-repair using a cone technique. METHODS Between June 2007 and October 2012, 134 patients underwent cone repair. Prior TV repair was performed in 20 patients (10 female, 50%). Median age was 15 years (range, 4 to 68 years). Four patients (20%) had prior bidirectional cavopulmonary anastomosis (BDCPA). Preoperative heart failure was present in 8 patients (40%). Recurrent tricuspid regurgitation (TR) was due to incomplete leaflet coaptation with tethered anterior leaflet in all patients; and 10 patients (50%) had diminutive septal leaflet. Prior repair techniques consisted of annuloplasty maneuvers in all patients with no or incomplete surgical delamination in all patients. RESULTS The TV re-repair using cone technique was possible in all patients. Plication of atrialized right ventricle was done in 6 patients (30%). Modifications included annuloplasty band in 16 patients (80%) and TV leaflet augmentation in 8 (40%). The BDCPA was performed in 3 patients (15%) and modified Cox-maze procedure was done in 10 (50%). There were no mortalities or reoperations. Mean follow-up was 7.7 ± 10.7 months; during follow-up, 18 patients had no or mild TR and 2 had moderate TR. CONCLUSIONS TV re-repair in EM using the cone technique is feasible, particularly when there has been no surgical leaflet delamination at initial operation. Leaflet augmentation facilitates and annuloplasty band stabilizes a competent TV re-repair. Longer follow-up is required to assess durability.

Fontan Conversion: Identifying the High-Risk Patient

BACKGROUND Patients with atriopulmonary Fontan tend to undergo conversion to total cavopulmonary connections secondary to arrhythmias or poor flow dynamics. However, the ideal candidate is unknown. METHODS Between December 1994 and May 2011, 70 patients (40 males [57%]) underwent Fontan conversion. Median age was 23 years (range, 4 to 46 years). Excluded were 1.5 ventricle conversions. The most common diagnoses included tricuspid atresia in 34 patients (49%) and double-inlet left ventricle in 16 (23%). Atrial tachyarrhythmia was present in 62 patients (89%), 41 (59%) had atrioventricular valve (AVV) regurgitation, and 32 (46%) were in New York Heart Association class III or IV. Atriopulmonary Fontan was the original connection in 58 patients (83%), whereas the Björk modification was performed in 8 (11%). RESULTS Fontan was performed with an intraatrial conduit in 41 patients, an extracardiac conduit in 18, and a lateral tunnel in 11. Forty-nine patients (70%) underwent concomitant arrhythmia operations. Early death occurred in 10 patients (14%). Multivariate analysis revealed age older than 27 years (p = 0.009), AVV regurgitation (p = 0.016), lack of arrhythmia operation (p = 0.04), and male sex (p = 0.02) were predictors of perioperative death. Mean follow-up was 5 years (maximum, 17 years). Overall survival at 1, 5, and 10 years was 81%, 70%, and 67%, respectively, and 84% of patients were in New York Heart Association class I or II. CONCLUSIONS Proper selection of Fontan conversion candidates is critical. Concomitant arrhythmia operations may be associated with improved survival. Older age and AVV regurgitation increase the risk of poor outcome, and cardiac transplantation may be a better option.

Cone Reconstruction in Children with Ebstein Anomaly: The Mayo Clinic Experience

OBJECTIVE Valve repair for pediatric patients with Ebstein anomaly has historically yielded varied results. The cone reconstruction (CR) first described by Da Silva has revolutionized the surgical approach to these patients. This study reports our recent experience with CR in children and young adults with Ebstein anomaly. DESIGN Electronic medical records were reviewed for all patients < 21 years old who had surgery to repair Ebstein anomaly at Mayo Clinic Rochester between June 2007 and December 2012. Clinical data including preoperative demographics, intraoperative procedures, and postoperative outcomes were recorded. RESULTS Eighty-four patients initially had a cone reconstruction (54% male, mean age 10.1 ± 5.9 years). Indications for operation included cardiomegaly (42%), cyanosis (19%), and heart failure (19%). The preoperative echocardiogram demonstrated severe tricuspid regurgitation in 91% of patients. There was one early death and 3 early CR breakdowns requiring reoperation (2 re-repair, 1 tricuspid replacement). Eighty-two patients (98%) had successful CR at the time of hospital discharge. Patient age, gender, cardiopulmonary bypass time, and aortic cross-clamp time were not associated with early CR failure. Use of a partial or eccentric annuloplasty ring correlated with successful initial CR (P = .01). There have been no early CR breakdowns since 2010. Follow-up information was available for 77 patients (longest follow-up 6.5 years; mean 0.8 ± 0.2 years). The most recent postoperative echocardiogram demonstrated mild or no tricuspid regurgitation in 83%. Tricuspid stenosis (mean gradient > 5 mm Hg) was present in 6 patients. There was one late death (motor vehicle accident) and one late re-repair of the tricuspid valve 4 years after initial operation. CONCLUSIONS CR in children and young adults with Ebstein anomaly can be performed with low early mortality and excellent durability at short-term follow-up. CR represents an important surgical option for young patients. It is applicable to patients with a broad range of anatomic variability and precludes valve replacement in the vast majority. CR should be considered prior to the deleterious effects of chronic right ventricular volume overload and the development of systolic dysfunction, which hamper long-term prognosis. Therefore, early referral for surgical evaluation is recommended.

Renal nutcracker syndrome: Surgical options

Nutcracker syndrome is one of the abdominal venous entrapments, caused by compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Occasionally a retro-aortic left renal vein is compressed between the aorta and the vertebral body (posterior nutcracker syndrome). The renal vein distal to the compression is dilated and renal venous flow can be diverted toward the pelvis through an incompetent, refluxing, left ovarian or spermatic vein, in addition to drainage through retroperitoneal venous collaterals. In this article, we describe the different surgical and endovascular techniques that are used to treat this syndrome.

Surgical Management of Congenital (Non-Ebstein) Tricuspid Valve Regurgitation

Congenital tricuspid valve regurgitation (TR) is a relatively uncommon condition that includes a heterogeneous group of lesions with a unique management strategy. There are wide anatomic variations that lead to congenital TR in patients without Ebstein malformation. Possible etiologies may include primary valve abnormalities (eg, congenital absence of chordae) or other forms of tricuspid valve dysplasia as in congenitally unguarded tricuspid valve, and patients with pulmonary atresia and intact ventricular septum, which can be similar to Ebsteins valves or secondary regurgitation in association with other anomalies as in atrioventricular septal defects, right ventricular outflow tract obstructive lesions (pulmonary stenosis or atresia with ventricular septal defect [VSD]), tricuspid valve annular dilatation in association with right ventricular volume overload lesions as in congenital coronary arterial fistula with secondary right ventricular enlargement, and Uhls anomaly. Iatrogenic etiologies in the congenital population include TR secondary to previous VSD closure (chordal or leaflet injury), pacemaker or internal cardiac defibrillator lead-induced TR, and traumatic TR (ruptured chordae). Presentation depends on the severity of the disease and may be apparent in infancy, childhood, or adulthood.

Bulging Subaortic Septum: An Important Risk Factor for Systolic Anterior Motion After Mitral Valve Repair

BACKGROUND The purpose of this study is to determine etiologic factors of systolic anterior motion (SAM) of the mitral valve after repair. METHODS We describe 6 patients with mitral valve repair (MVRep) for degenerative mitral valve disease and a bulging angulated subaortic septum that seemed a risk factor for SAM. RESULTS No patient had a hypertrophic cardiomyopathy diagnosis. All patients had an angulated septum (angle of long axis of left ventricle and aorta, >60°) with a discrete septal bulge (proximal septum, ≥1.5 cm with normal midseptal thickness). One patient had severe mitral regurgitation and became hypotensive intraoperatively, with a SAM. He had successful valve repair and septal myectomy. In 2 patients, the bulging septum was not appreciated preoperatively, and SAM developed postoperatively, causing symptomatic left ventricular (LV) outflow tract (LVOT) obstruction and recurrent mitral regurgitation. At reoperation, SAM and associated regurgitation were abolished with septal myectomy. In 2 patients undergoing mitral repair, bulging septum was identified preoperatively, and prophylactic septal myectomy was performed at valve repair. One patient was referred for valve replacement because of severe mitral regurgitation due to bileaflet prolapse and associated SAM. Doppler echocardiography showed minimal resting gradient. He had a prominent subaortic septum, and mitral regurgitation seemed due to organic valve disease and dynamic outflow obstruction. He received septal myectomy and mitral valve repair with resolution of SAM and mitral regurgitation. CONCLUSIONS Bulging subaortic septum may be a risk factor for SAM after mitral valve repair. Treatment should include septal myectomy with valvuloplasty.

Late outcome of repair of congenital coronary artery fistulas—a word of caution

OBJECTIVES We reviewed our experience with repair of congenital coronary artery fistulas. METHODS From June 1983 to December 2009, 46 patients (median age, 59 years; range, 1-84 years) underwent surgical repair. The presenting symptoms included angina in 16 patients (35%), congestive heart failure in 11 (24%), and bacterial endocarditis in 5 (11%). Preoperatively, 9 patients (20%) had at least moderate tricuspid regurgitation. Coronary artery dominance was right in 38 patients (83%). Coronary artery aneurysms were found in 8 patients (17%). The most common pattern was right coronary artery-to-coronary sinus fistula (18 patients, 39%); 11 patients had (23%) more than 1 fistula. One patient had undergone previous coil embolization. RESULTS Cardiopulmonary bypass was used in 39 patients (85%), with extracardiac and intracardiac repair performed in 30 (65%) and 16 (35%), respectively. The most common associated procedures were coronary artery bypass in 13 patients (28%). Early mortality occurred in 1 patient (2%). Postoperative myocardial infarction occurred in 5 patients (11%); 4 of these patients underwent simple ligation or division of their fistulas. The mean follow-up was 6 ± 5.8 years (maximum, 22 years). Late mortality occurred in 11 patients (24%). Two patients underwent reoperation for severe tricuspid regurgitation. Survival was significantly reduced compared with the age- and gender-matched population (P = .03). Residual fistulas were detected in 3 patients (6%), with no reintervention needed. CONCLUSIONS Perioperative myocardial infarction is an important complication of ligation of coronary artery fistulas and can contribute to reduced late survival. The tricuspid valve should be evaluated carefully at repair because of the relatively high rate of residual regurgitation in survivors.

Do Pericardial Bioprostheses Improve Outcome of Elderly Patients Undergoing Aortic Valve Replacement

BACKGROUND Pericardial bioprostheses have favorable echocardiographic hemodynamics in the aortic position compared with porcine valves; however, there are few data comparing clinical outcomes. Our objective was to assess the late results of the two valve types. METHODS We reviewed 2,979 patients aged 65 years or older undergoing aortic valve replacement with pericardial (n=1,976) or porcine (n=1,003) prostheses between January 1993 and December 2007. The most common pericardial prostheses were Carpentier-Edwards Perimount and Mitroflow, and the most common porcine valves were Medtronic Mosaic, Carpentier-Edwards, Hancock modified orifice, and St. Jude Biocor. Follow-up extended to a maximum of 16 years (mean, 5.2±3.5 years). RESULTS Survival at 5, 10 and 12 years was, respectively, 68%, 33%, and 21% overall, was 68%, 30%, and 16% for patients with pericardial bioprosthesis, and was 69%, 38% and 27% for the porcine group. In a multivariate model, long-term survival was reduced in patients with diabetes, renal failure, prior myocardial infarction, congestive heart failure, and older age, but late survival was not higher in the pericardial valve group. Overall freedom from reoperation was 96%, 92%, and 90% at 5, 10, and 12 years, and freedom from explant was 98%, 96%, and 94% during the same period. The reason for explant was structural valve deterioration in 50 patients (2%). CONCLUSIONS Despite the better hemodynamic performance documented in prior investigations, pericardial valves do not confer any survival advantage over porcine valves in patients aged 65 years or older undergoing aortic valve replacement.

Pulmonary embolism after endovenous thermal ablation of the saphenous vein

Pulmonary embolism (PE) after venous procedures is fortunately rare. Our goal was to analyze the data of patients who developed PE after endovenous thermal ablation and phlebectomy for varicose veins and to review the literature on this subject. We report on three patients who developed PE after radiofrequency ablation of the great saphenous vein and mini phlebectomy for symptomatic primary lower-extremity varicose veins. Early postoperative duplex scans confirmed successful closure of the great saphenous vein in all. One patient presented with chest pain and dyspnea, one with blood-tinged sputum, and the third with symptoms of saphenous thrombophlebitis. Two patients had PE from the saphenous vein thrombus and the third had gastrocnemius vein thrombosis extending into the popliteal vein. One had previous deep vein thrombosis. Computed tomography of the chest confirmed PE in all. Two patients were treated with anticoagulation, but the third patient with small PE declined such treatment. One patient underwent temporary inferior vena cava filter placement because of recurrent PE. In conclusion, PE is very rare but it can occur after endovenous thermal ablation of lower-extremity varicose veins. Selective thrombosis prophylaxis and preoperative counseling of the patients about signs and symptoms of deep vein thrombosis and PE are warranted for early recognition and rapid treatment.