Percutaneous mitral valve repair in recurrent severe mitral valve regurgitation after mitral annuloplasty : MitraClip-in-the-ring as a complementary strategy.

BACKGROUND: Patients with reduced left ventricular (LV) function undergoing coronary artery bypass graft surgery or/and aortic valve replacement occasionally show severe mitral valve (MV) regurgitation and thus also undergo surgical mitral annuloplasty. Over time, further deterioration of LV function and additional ischemic events cause recurrence of severe MV regurgitation due to the Carpentier IIIb morphology of the MV that is not adequately addressed by the previously implanted annuloplasty ring. METHODS: Seven patients (Society of Thoracic Surgeons score: 7.5 ± 1.5%) with Carpentier type-IIIb recurrent severe MV regurgitation, having undergone prior cardiothoracic surgery (median: 40 months) including mitral annuloplasty, were treated with the MitraClip device. RESULTS: MitraClip implantation resulted in significantly reduced MV regurgitation and improved New York Heart Association functional state, translating into an increased exercise capability and improved cardiac biomarkers. The morphology of the MV was adequately addressed without causing relevant MV stenosis, while the MV annulus area remained unaltered. The procedure was safe with a 30-day mortality rate of 0%. CONCLUSION: MitraClip-in-the-ring is feasible and in principle safe for treating Carpentier type IIIb severe MV regurgitation after surgical MV repair using mitral annuloplasty. MitraClip-in-the-ring resulted in immediate amelioration of clinical symptoms and increased physical exercise capacity.

A rare, combined cardiac and hepatic crossbow injury.

A 32-year-old man presented to our hospital after a failed suicide attempt using a crossbow. The patient had shot himself in close range with a double-barreled crossbow, simultaneously two arrows, in the chest. In spite of his life-threatening injuries, the man survived. The lodged arrows helped to avoid tremendous loss of blood. After his arrival in the hospital, the surgical removal of the arrows proceeded under direct visualization with corresponding sutures on the heart and liver. The patient's postoperative course was uneventful.

Perioperative management of a child with glutaric aciduria type I undergoing cardiac surgery.

Patients with glutaric aciduria type I are at risk for acute striatal injury precipitated by catabolic stress. Here, we report the successful interdisciplinary anesthetic and perioperative management of a child with glutaric aciduria type I undergoing cardiac surgery with extracorporeal circulation. Given the central focus on prevention of acute striatal injury, our anesthetic strategy emphasized avoiding a high protein load, high-dose inotropics, especially epinephrine (associated with impaired glucose utilization), deliberate hyperventilation, and other interventions associated with systemic inflammatory response.

Comparison of inhaled nitric oxide with aerosolized iloprost for treatment of pulmonary hypertension in children after cardiopulmonary bypass surgery.

OBJECTIVES: Pilot study to compare the effect of inhaled nitric oxide (iNO) and aerosolized iloprost in preventing perioperative pulmonary hypertensive crises (PHTCs). BACKGROUND: Guidelines recommend the use of iNO to treat PHTCs, but treatment with iNO is not an ideal vasodilator. Aerosolized iloprost may be a possible alternative to iNO in this setting. METHODS: Investigator-initiated, open-label, randomized clinical trial in 15 infants (age range 77-257 days) with left-to-right shunt (11 out of 15 with additional trisomy 21), and pulmonary hypertension (i.e. mean pulmonary artery pressure [PAP] >25 mmHg) after weaning from cardiopulmonary bypass. Patients were randomized to treatment with iNO at 10 ppm or aerosolized iloprost at 0.5 µg/kg (every 2 h). The observation period was 72 h after weaning from cardiopulmonary bypass. The primary endpoint was the occurrence of PHTCs; the secondary endpoints were mean PAP, duration of mechanical ventilation, safety of administration, and in-hospital mortality. RESULTS: Seven patients received iNO and eight patients received iloprost. During the observation period, 13 of the 15 patients had at least one major or minor PHTC. There was no difference between the groups with regard to the frequency of PHTCs, mean PAP and duration of mechanical ventilation (p > 0.05). CONCLUSIONS: In this pilot study, aerosolized iloprost had a favorable safety profile. Larger trials are needed to compare its efficacy to iNO for the treatment of perioperative pulmonary hypertension. However, neither treatment alone abolished the occurrence of PHTCs.

Endothelial nitric oxide synthase gene polymorphism (Glu298Asp) and acute pulmonary hypertension post cardiopulmonary bypass in children with congenital cardiac diseases.

BACKGROUND: Intra-cardiac repair of congenital cardiac diseases in children with left-right shunt is often associated with acute elevation of pulmonary artery pressure following cardiopulmonary bypass. We studied the correlation between the Glu298Asp polymorphism of the endothelial nitric oxide synthase gene and pulmonary hypertension in children with congenital cardiac diseases. METHODS AND RESULTS: A total of 80 children with congenital cardiac diseases at a median age of 3.8 years, ranged 0.1-36.2 years, and 136 controls were enrolled. Most patients presented with significant left-to-right shunt - pulmonary-to-systemic blood flow of 2.8, with a range from 0.6 to 7.5. In all, 40 out of 80 children showed pulmonary hypertension with mean pressure of 42, ranged 26-82, millimetres of mercury. Thirty-one out of 40 children underwent intra-cardiac repair and 15 out of 31 operated patients were found to have an acute elevation of pulmonary artery pressure after cardiopulmonary bypass. The Glu298Asp polymorphism was identified using polymerase chain reaction and restriction fragment length polymorphism. Both in patients and in controls, the genotype distribution corresponded to the Hardy-Weinberg equilibrium. The gene frequency for Glu298Glu, Glu298Asp and Asp298Asp was not different in the control group compared to the patients (Armitage trend test: p = 0.37). The endothelial nitric oxide synthase polymorphism was related to acute post-operative elevation of pulmonary artery pressure (genotypic frequency 53.3 versus 25%; Armitage trend test: p = 0.038). In addition, the allelic frequency of the Glu298Asp was related to post-operative pulmonary hypertension (Fischer's exact test: p = 0.048). The positive predictive value was 71.43%. CONCLUSION: Patients with left-to-right shunt are more likely to develop acute elevation of pulmonary artery pressure after cardiopulmonary bypass when presenting with the Glu298Asp polymorphism of the gene endothelial nitric oxide synthase. This could be used as a genetic marker for the predisposition for the development of pulmonary hypertension after intra-cardiac repair.

Long-term outcome after external tracheal stabilization due to congenital tracheal instability.

BACKGROUND: Long-segment tracheobronchial malacia may cause life-threatening dysfunction of the airway system at different levels. This study presents the long-term follow-up (1992 through 2008) of patients who received surgical treatment with external tracheal stabilization in our institution. METHODS: Eleven patients fulfilled the inclusion criteria. In surviving patients who presented for reexamination, pulmonary function testing, ergometry, and magnetic resonance imaging (MRI) were performed. RESULTS: All patients could be weaned from the ventilator and discharged. Patients were aged a median 11 months (range, 3 to 48 months) at operation for tracheal compression. Age at follow-up was 9.1 years (range, 0.5 to 16.3 years). Median follow-up was 7.3 years (range, 0.1 to 15.1 years). Postoperatively, 1 patient was lost to follow-up, and 4 died at 2.6 years (range, 0.5 to 6.6 years) of comorbidities. Pulmonary function testing showed a moderate residual airflow restriction, with maximal vital capacity at 75% of normal (range, 45% to 92%). Treadmill exercise testing demonstrated 70% to 89% of the expected normal values for age. Magnetic resonance imaging examination confirmed tracheal patency, but the lumen of the left main bronchus in 2 patients was 50% smaller than on the right. Diaphragmatic motion was normal in all patients. CONCLUSIONS: Children with congenital tracheal stenosis benefit from external tracheal stabilization. Survival in patients after external tracheal stabilization is significantly influenced by concomitant conditions.

Expression and mutational status of PDGFR in thymic tumours.

BACKGROUND: There is an ongoing search for new therapeutic targets in invasive non-resectable thymic tumours because of the low response rates in current chemotherapeutic treatment modalities. In this study, the possibility that platelet-derived growth factor receptor A (PDGFRA) and/ or PDGFRB may represent potential therapeutic targets in epithelial tumours of the thymus was investigated. PATIENTS AND METHODS: Tissue samples were obtained by thymectomy from 36 different patients with epithelial tumours of the thymus (26 thymomas types A, AB, B1-3 and 10 thymic carcinomas). Normal thymi from three young children were used as controls. The PDGFRA and PDGFRB protein expressions as well as the mutational statuses of exons 12, 14 and 18 of the PDGFRA gene were analyzed. RESULTS: All the subtypes of thymomas and the thymic carcinomas showed staining for PDGFRA, but no mutations in the known mutational hotspots were identified. Only about one third of the tumours stained for PDGFRB. PDGFRA and PDGFRB protein staining were slightly positively correlated. CONCLUSION: PDGFRA may represent a potential therapeutic target in thymic tumours.

The evolution of the pulmonary arterial sling syndrome, with particular reference to the need for reoperations because of untreated tracheal stenosis.

BACKGROUND: We present a group of infants and children with pulmonary arterial sling and tracheal stenosis. In some of the patients, the anomalously located pulmonary artery had previously been reimplanted, but without simultaneous repair of the trachea. METHODS: From 1992 to 2007, we reimplanted the left pulmonary artery in 13 children with a pulmonary arterial sling. Their median age was 8 months, with a range from 1 to 72 months. We also performed tracheal resection with end-to-end anastomosis, or complex tracheal reconstructions. In 5 patients, the reoperation was indicated because of persistent tracheal stenosis not treated initially at first correction of the arterial sling. All patients presented with stridor and respiratory distress. Cardiac catheterization, bronchoscopy and multidetecting computer tomography angiography were performed in all cases prior to the operation. All operations were performed under cardiopulmonary bypass. RESULTS: There was no operative or late mortality. The patients were extubated under bronchoscopic control. The mean period of intubation was 18 plus or minus 8 days, and the average follow-up was 8 plus or minus 4 years. The patients showed no signs of tracheal re-stenosis clinically or on bronchoscopy. The group of the patients under reoperations, however, required longer periods of intubation and hospitalization. CONCLUSION: Our experience demonstrates that, in patients with a pulmonary arterial sling, any associated tracheal stenosis should be explored at the initial operation, since decompression of the trachea by reimplanting the anomalously located pulmonary artery fails to provide relief. The funnel trachea, if present, undergoes progressive stenosis, and will require surgical repair. The use of cardiopulmonary bypass permitted extensive mobilization of the tracheobronchial tree, and allowed us to perform a tension-free anastomotic reconstruction of the trachea.

Endothelin-1 and asymmetric dimethylarginine in children with left-to-right shunt after intracardiac repair.

BACKGROUND: Endothelin-1 (ET-1) is an endogenous vasoconstrictive peptide hormone and asymmetric dimethylarginine (ADMA) acts as an endogenous inhibitor of nitric oxide synthase. We hypothesized that both could contribute to pulmonary hypertension in patients with left-to-right shunt after intracardiac repair. PATIENTS AND METHODS: We prospectively analyzed ET-1 and ADMA plasma levels in 31 patients (m = 16; f = 15) at an age of 0.6 [0.2-27] years (median [range]) with left-to-right shunt (ASD II: n = 12; VSD: n = 11; AVSD: n = 8) presenting with a Qp/Qs of 2.7 [1.4-6.3] and a pulmonary arterial mean pressure (PAP) of 23 [13-57] mmHg. Blood specimens were taken prior to cardiopulmonary bypass (CPB), after weaning from CPB and at 3, 6, 12 and 24 h after CPB. RESULTS: 12/31 patients were found to have pulmonary hypertension prior to intracardiac repair and 11/12 patients showed persistent pulmonary hypertension during the first 24 h after CPB. Patients with pulmonary hypertension at 12 h after CPB showed significant higher plasma ET-1 compared with patients with normal PAP (1.4 [0-7.9] versus 0.5 [0-2.5] pg/ml; P = 0.048 (Mann-Whitney)). Plasma ADMA decreased from 1.3 [0.75-2.3] micromol/l before CPB to 0.7 [0.4-2.1] micromol/l at 12 h (P < 0.05). However patients with pulmonary hypertension did not show different ADMA plasma levels. CONCLUSIONS: Increased plasma ET-1 but not inhibition of nitric oxide synthase by ADMA is associated with pulmonary hypertension after intracardiac repair.

Modified sliding tracheal plasty using the bridging bronchus for repair of long-segment tracheal stenosis.

A child with severe respiratory distress, previously operated on at age 4 months for pulmonary sling and atrial septal defect, underwent reoperation at age 4 years because of long-segment congenital tracheal stenosis complicated by an abnormal branching of the trachea-bridging bronchus. We review the anatomy of that rare pathomorphology and describe a slide tracheoplasty that uses the bridging bronchus for treatment of this complex anomaly.

Outcome after mechanical aortic valve replacement in children and young adults.

BACKGROUND: We asked whether aortic valve replacement using a mechanical prosthesis would allow normalization of left ventricular function and structure in children and young adults. METHODS: We performed a clinical follow-up examination in 30 patients with aortic valve replacement at 25 years of age or younger, including conventional and tissue Doppler echocardiography and magnetic resonance imaging. RESULTS: Aortic valve replacement was performed at the median age of 14.3 years (range, 7.6 to 24.3 years) using a mechanical prosthesis (St. Jude Medical; median diameter, 23 mm; range, 17 to 27 mm). Indications were severe aortic stenosis in 6 of 30 patients, aortic regurgitation in 20 of 30 patients, or a combination of aortic stenosis and regurgitation (4 of 30 patients). Aortic valve replacement was a reoperation in 12 of 30 patients who primarily underwent aortic valvotomy at a median of 7.1 years (range, 1.0 to 11.3 years). In-hospital mortality was 0%. Follow-up was a median of 6 years (range, 1.2 to 14.5 years). Twenty-nine of 30 patients were in New York Heart Association functional class I without thromboembolic complications, cerebrovascular accidents, or major bleeding on oral anticoagulation. Left ventricular dilatation before aortic valve replacement was present in 20 of 30 patients but normalized in all but 4 patients on follow-up. Most patients showed a normal end-diastolic volume on magnetic resonance imaging, and 23 of 26 patients showed a normal left ventricular ejection fraction (median, 0.53; range, 0.33 to 0.75). Peak systolic strain of the left ventricular myocardium was a median of -13.3% (range, -0.5% to -31%), and was normal in 28 of 30 patients. CONCLUSIONS: Aortic valve replacement in children and young adults offers a good treatment option and may lead to normalization of left ventricular size and function in most patients.

Replacement of valved right ventricular to pulmonary artery conduits: an observational study with focus on right ventricular geometry.

OBJECTIVE: Timing of the operation for exchange of right ventricular (RV) to pulmonary artery (PA) conduits is a matter of considerable debate. We aimed to study the course of right ventricular dimension in patients undergoing conduit exchange. PATIENTS AND METHODS: We retrospectively studied all patients who underwent implantation and or replacement of RV/PA conduits during the time period between 1990 and 2005. Clinical and echocardiographic data were recorded as obtained at follow-up visits. RESULTS: A total of 229 (144 boys and 85 girls) underwent surgery for implantation and or replacement of RV/PA conduits during the study period. Patients were assigned to three age groups including 37 infants, 125 children aged 1-10 years and 67 patients more than 10 years of age. 185 pulmonary (81%) and 44 aortic homografts (19%) were implanted. Fifty-eight of these 185 patients (25%) required exchange of conduits after a median time of 6.4 (8 months-12 years) (median (range)). The follow-up was 7.55 (0.1-17) years. The survival of the patients after homograft change was 98%. Freedom from failure for aortic and pulmonary homografts at an interval of 10 years for all patients was 38.5% for aortic and 56.2% for pulmonary homografts (P = 0.018; Mann-Whitney). Age at conduit exchange (coefficient: -4.917; P < 0.001) and right ventricular end-diastolic dimension (RVDD) before conduit exchange (coefficient: 8.255; P < 0.001) were related to RVDD as measured by M-mode echocardiography at follow-up ("best subset" regression analysis; R squared = 0.746). RVDD decreased in 48/58 patients, remained unchanged in 8/58 and increased in 2/59 patients at follow-up. An increased RVDD was positively correlated to the duration of artificial ventilation after the operation for conduit exchange (R = 0.56; P < 0.001). CONCLUSIONS: Reoperation for exchange of degenerated conduits should be performed early to prevent the development of irreversible structural myocardial changes and persistence of right ventricular dilatation.

Late results after extended pulmonary artery reconstruction in the arterial switch operation.

BACKGROUND: Pulmonary artery stenosis remains the most frequent late complication and cause of reintervention after the arterial switch operation for transposition of the great arteries. We investigated the influence of an extended pericardial patch augmentation of the neopulmonary root and pulmonary artery on late pulmonary artery stenosis development. METHODS: Augmentation of the neopulmonary root and pulmonary artery was achieved by reconstructing the posterior wall using a large glutaraldehyde-treated autologous pericardial patch. Reviewed were regular follow-up echocardiograms from 58 out of 87 patients undergoing the arterial switch operation who presented a follow-up period of at least 5 years. An actual follow-up echocardiographic evaluation focusing on the maximal instantaneous transpulmonary continuous-wave (cw)-Doppler gradient was performed, followed by cardiac catheterization when indicated (peak cw-Doppler gradient > 40 mm Hg). RESULTS: Follow-up was 8.9 [5 to 15] years. There was no reintervention due to residual pulmonary artery stenosis. Actual Doppler examination revealed a transpulmonary peak gradient of 19.5 [0 to 56] mm Hg, compared with 20 [0 to 60] mm Hg at discharge. Forty-three patients (74.1%) had no or only trivial pulmonary artery stenosis (pressure gradient < 25 mm Hg), 14 patients (24.2%) had mild stenosis (25 to 49 mm Hg), and 1 patient (1.7%) had moderate stenosis (50 to 79 mm Hg). CONCLUSIONS: Compared with the majority of literature data, we could demonstrate a low incidence of late pulmonary artery stenosis after the arterial switch operation by employing an extended pericardial patch reconstruction technique with augmentation of the neopulmonary root and pulmonary artery.

Anomalous connection of the right superior caval vein to the morphologically left atrium.

Anomalous drainage of the right superior caval vein into the morphologically left atrium as an isolated cardiac malformation is a rare anomaly. Most patients present with cyanosis. Thus far, about 20 cases have been reported in the literature. We report a case of cyanosis due to this malformation in a male neonate, which was complicated by the meconium aspiration syndrome. The malformation was diagnosed by echocardiography and cardiac catheterization. Surgery resulted in complete recovery.

Simultaneous management of congenital tracheal stenosis and cardiac anomalies in infants.

OBJECTIVE: The present article aims to describe our experience with patients who underwent simultaneous repair of congenital tracheal stenosis and cardiac anomalies. METHODS: Between January 2000 and December 2003, 9 infants underwent simultaneous surgical repair of a congenital tracheal stenosis and congenital heart disease. The intraoperative findings revealed localized tracheal stenosis in 3 patients. The funnel-type tracheal stenosis was present in 6 patients. Associated cardiac anomalies included ostium secundum atrial septal defect in 5 patients and ventricular septal defect in 2 patients, pulmonary artery sling in 4 patients, patent ductus arteriosus in 6 patients, atrioventricular septal defect in 1 patient, aortic arch hypoplasia in 1 patient, coarctation of the aorta in 1 patient, and partial anomalous pulmonary venous connection in 2 patients, one of them with "scimitar syndrome." Tracheal origin of the right upper lobe was diagnosed in 2 of the patients. A right aberrant subclavian artery (lusoria) was found in one patient. All patients were operated on through a median sternotomy and with cardiopulmonary bypass. Tracheal resection with direct end-to-end anastomosis was performed in all cases. RESULTS: There was no operative mortality. One patient died 6 weeks postoperatively. Eight patients were extubated between the 14th and 30th postoperative days under bronchoscopic monitoring. The extubation was performed after a stepwise respirator-weaning program. Postoperative endoscopic examination showed adequate airway dimensions and patency in every case. The midterm results after a mean follow-up of 37 months (range, 16-58 months) of the entire group demonstrate a stabile and complication-free clinical outcome. CONCLUSIONS: We advocate our current strategy for infants with congenital tracheal stenosis: resection with end-to-end anastomosis and simultaneous repair of associated intracardiac anomalies.

A case of pulmonary artery sling associated with long-segment funnel trachea and bronchus suis.

An infant with pulmonary sling syndrome associated with an abnormal branching of the trachea, bronchus suis, complicated by long segment intrathoracic tracheal stenosis (ie, "funnel trachea") and concomitant tracheomalacia underwent reoperation at 5 months of age. We review the anatomy of this rare entity and describe the surgical technique to avoid the residual stenosis.

Ventricularization of the atrialized chamber: a concept of Ebstein's anomaly repair.

BACKGROUND: We report results of a technique of Ebstein's anomaly repair by creating a predominantly monocuspid valve with simultaneous ventricularization of the atrialized right ventricular (aRV) chamber. METHODS: Between March 1993 and April 2003, Ebstein's anomaly repair by valvuloplasty with combined ventricularization was performed in 23 patients aged 13.6 (4.1-52.6) years presenting with tricuspid valve regurgitation (TVR) (I degrees, n = 1; II degrees, n = 3; III degrees, n = 13; IV degrees, n = 6). Valvuloplasty consisted of creating a predominantly monocuspid valve at the level of the anatomical atrioventricular junction resulting in a ventricularization of the atrialized chamber. Postoperatively all survivors were examined regularly with an actual prospective evaluation. RESULTS: One early death (4.4%) occurred and was caused by right heart failure. Follow-up was 4.6 (0.5-10.9) years. Important recurrent atrioventricular valve regurgitation caused by rupture of fixation sutures occurred in 3 patients (13%), necessitating reintervention at 3 (0.03-4) months (revalvuloplasty, n = 2; TV replacement, n = 1). One patient presenting with hypoplastic right ventricle with consecutive right heart failure underwent creation of a total cavopulmonary connection at 10 months. At present all patients are doing well. Actual echocardiographic examination revealed significant improvement of right atrioventricular valve regurgitation (p < 0.0001) and favorable restoration of RV geometry and function. CONCLUSIONS: This technique of Ebstein's anomaly repair with ventricularization of the atrialized chamber provides excellent results regarding right atrioventricular valve function and leads to a favorable restoration of RV geometry and function.

Long-term results after reconstruction of the left ventricular outflow tract by aortoventriculoplasty.

BACKGROUND: Aortoventriculoplasty is an established method of reconstruction of complex left ventricular outflow tract (LVOT) obstruction by insertion of a mechanical valve prosthesis after patch enlargement of the LVOT. Little data exist with respect to long-term outcome. METHODS: Between March 1991 and June 2001, 24 patients with a median age of 10.7 (range, 2.1 to 66) years underwent aortoventriculoplasty, which was performed as a primary procedure in 4 and as a secondary intervention in 20 patients. On follow-up, all patients were restudied with an actual prospective evaluation. Data were statistically analyzed using a paired t test. RESULTS: There was one early death from low cardiac output. Four patients had to be reoperated on for bleeding. All 23 postoperative survivors were followed up for 63 (range, 12 to 123) months. Aortic anulus size of 14 (7 to 19) mm could be significantly enlarged to a size of 24 (19 to 27) mm for insertion of a mechanical valve prosthesis (p < 0.001). Blood flow velocity across the LVOT significantly decreased to 1.8 (1.3 to 2.9) m/s (preoperative, 4.1 [2.7 to 5.8] m/s) (p < 0.001). There were no late deaths. One patient underwent late repair of a paraprosthetic leak. On follow-up, there was no hemorrhage related to anticoagulation observed, but there was one minor thromboembolic complication. Relief of LVOT obstruction and good function of the valve prostheses could be demonstrated in all patients. CONCLUSIONS: Aortoventriculoplasty is an easily applicable, low-risk procedure for the effective relief of complex LVOT obstruction, and provides excellent long-term results.