Cone versus conventional repair for Ebstein's anomaly.

OBJECTIVES: We aimed to investigate tricuspid valve function and adverse events after conventional repair and valve replacement for Ebstein's anomaly and compare them with cone repair. METHODS: The medical records of 151 patients (mean age, 25 years; 62% were female) who underwent operation in a single center from 1985 to 2018 were retrospectively analyzed. To determine tricuspid valve regurgitation during follow-up, serial echocardiographic examination was used (n = 2397, tricuspid regurgitation grades were graphed for every patient). RESULTS: Thirty-nine patients underwent cone repair, 107 patients underwent other repair techniques, and 5 patients underwent valve replacement. The operative mortality was 1.3% (n = 2). Failed valve repair (defined as in-hospital death, conversion to replacement, or in-hospital reoperation) was less frequent after cone repair than after other repair techniques (5%, n = 2 vs 20%, n = 21, P = .039). Mean follow-up was 12.3 years (cone repair: 3.7 years). The 5-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation was lower after cone repair than after other repair techniques (8% vs 32%, P = .03). Among the patients undergoing other repair techniques, the 15-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation, severe tricuspid regurgitation, and reoperation was 58%, 37%, and 31%, respectively. During follow-up, 18 patients died (13 of cardiac and 5 of noncardiac causes). Among patients who died of cardiac causes, 10 of 13 had all 3 characteristics-moderate or greater tricuspid regurgitation, atrial fibrillation, and New York Heart Association classification III and IV-at their last medical evaluation. CONCLUSIONS: Before cone repair, recurrent tricuspid regurgitation was considerable. Cone repair provided a higher rate of successful repair and a lower incidence of moderate or greater recurrent tricuspid regurgitation at the midterm follow-up.

Impact of Characteristics at Stage-2-Palliation on Outcome Following Fontan Completion.

The optimal timing of stage-2-palliation (S2P) in single left ventricle is not clear. The aim of this study was to identify S2P related factors associated with outcomes after total cavopulmonary connection (TCPC), particularly relative to the dominant systemic ventricle. A total of 405 patients who underwent both S2P and TCPC at our institute between 1997 and 2017 was included. Patients were divided into two groups, dominant right ventricle (RV type, n = 235) and dominant left ventricle (LV type, n = 170). S2P related factors associated with mortality, postoperative ventricular function, and late exercise capacity following TCPC, were analyzed. The median age at S2P was 4 [3-7] and 6 [3-11] months in RV and LV type patients, respectively (p = 0.092). Survival after TCPC was similar in RV and LV type patients (p = 0.280). In those with RV type, risk factors for mortality following TCPC were older age (p < 0.001), heavier weight (p = 0.001), higher PAP (p < 0.001), higher TPG (p = 0.010), and lower SO2 (p = 0.008) at S2P. In those with LV type, no risk factor was identified. Risk factors for postoperative impaired ventricular function were older age and higher weight at S2P in both RV and LV type patients. Older age at S2P was also identified as a risk for inferior peak oxygen uptake (VO2) years after TCPC both in RV and LV type patients. Older age at S2P was associated with higher mortality after Fontan completion only in RV type patients. However, it was associated with postoperative ventricular dysfunction and lower exercise capacity after TCPC in both RV and LV type patients.

Reasons for Failure of Systemic-to-Pulmonary Artery Shunts in Neonates.

BACKGROUND: Systemic-to-pulmonary artery shunt placement is an established palliative procedure for congenital heart disease. Although it is thought to be a simple operation, it is associated with significant morbidity and mortality. METHODS: Data for all neonates who underwent surgery for a systemic-to-pulmonary artery shunt between 2000 and 2016 were reviewed. The study endpoints were shunt failure and shunt-related mortality. Shunt failure was defined as a shunt dysfunction because of thrombosis or stenosis requiring intervention or reoperation; shunt mortality was defined as death because of a shunt dysfunction. RESULTS: A total of 305 shunts (central shunt, n = 135; Blalock-Taussig shunt, n = 170) were implanted in 280 patients. The median patients' age at the time of surgery was 9 days (1-31 days). The median shunt size was 3.5 mm (3-4 mm). Twenty-four patients (8%) were diagnosed with a shunt failure, with a median time of 7 days (0-438 days). Freedom from shunt failure at 1 year was 91.6% ± 2%. A shunt-related mortality was ascertained for 12 patients (4%). Freedom from shunt-related mortality at 1 year was 96% ± 1%. Perioperative platelet transfusion (p = 0.01), central shunt (p = 0.02), 3-mm shunt size (p = 0.02), and postoperative extra corporeal membrane oxygenation (ECMO) (p < 0.01) were identified as risk factors for shunt failure. Platelet transfusion (p = 0.04) and postoperative ECMO (p < 0.01) were further identified as risk factors for shunt mortality. CONCLUSION: Based on these data, we recommend implanting a modified Blalock-Taussig shunt of at least 3.5 mm in neonates. Perioperative platelet transfusion and postoperative ECMO increase the risk of shunt failure.

Predicted clinical factors associated with the intensive care unit length of stay after total cavopulmonary connection.

OBJECTIVES: A longer length of stay (LOS) in the intensive care unit (ICU) after the total cavopulmonary connection (TCPC) is thought to be a predictive sign of late Fontan failure. This study was performed to determine the clinical risk factors for ICU LOS. METHODS: In total, 483 patients who underwent a TCPC between May 1994 and December 2016 were included the study. Patients' main diagnosis, morphologic characteristics, palliative procedures, hemodynamic parameters, and perioperative variables, were analyzed to identify risk factors influencing ICU stay based on Cox regression. Causes of longer ICU LOS and the impact of ICU LOS on late outcomes were evaluated. RESULTS: Age at TCPC, type of TCPC, and fenestration at TCPC did not affect the ICU LOS. With multivariable model, hypoplastic left heart syndrome (P = .001) and anomalous systemic venous drainage (P < .001) were identified as independent morphologic risk factors for prolonged ICU LOS. Of hemodynamic variables, preoperative high transpulmonary gradient (P = .037), and low aortic oxygen saturation (P = .031) were risks for longer ICU LOS. Of postoperative variables, pleural effusion (P < .001), chylothorax (P = .001), ascites (P < .001), and infection (P = .028) were risks for longer ICU LOS. The ICU LOS was found to be significantly associated with late mortality (P < .001) and late cardiac reoperation (P = .007). CONCLUSIONS: Patients with hypoplastic left heart syndrome and anomalous systemic venous drainage had longer ICU LOS. Extended cyanosis and elevated pulmonary artery pressure affect the ICU LOS. Special care should be provided during the initial postoperative phase in patients with such risk factors.

Preoperative predictability of right ventricular failure following surgery for Ebstein's anomaly.

OBJECTIVES: Postoperative right ventricular (RV) failure is a severe complication after tricuspid valve (TV) surgery in patients with Ebstein's anomaly. We investigated the preoperative predictability of postoperative mortality and morbidity by assessing the influence of age, RV size and RV function on in-hospital mortality and on the clinical course during the intensive care unit (ICU) stay. METHODS: We retrospectively analysed 189 patients who had undergone TV surgery for Ebstein's anomaly at our centre. For this study, only patients with preoperative cardiac magnetic resonance imaging (MRI) scans, who were operated on from 2005 to May 2018, were included. Three potential risk factors were proposed: (i) RV end-diastolic volume index >200 ml/m2, (ii) RV ejection fraction (EF) <40% and (iii) age at operation >50 years. Primary end points were death or the need for extracorporeal membrane oxygenation. Secondary end points were postoperative inotropic therapy, ventilation time, renal failure and duration of ICU stay and hospital stay. RESULTS: A total of 70 patients with preoperative cardiac MRI scans were included: 57 had undergone TV repair and 13 TV replacement. Thirty patients exhibited none of the defined risk factors, 24 patients exhibited 1 risk factor, 13 patients exhibited 2 risk factors and 3 patients exhibited 3 risk factors. There were 4 in-hospital deaths (6%): 3 of these patients had 3 risk factors, and 1 patient had 2 risk factors. In patients with 1 or more risk factors, the odds ratio for primary end point was 2.5 (P = 0.43) and in patients with 2 or more risk factors, the odds ratio was 18.5 (P = 0.001). Patients with at least 1 risk factor required prolonged inotropic drug administration and required a longer hospital stay (median 20 days vs 14 days, P = 0.029). Patients with at least 2 risk factors showed a significantly prolonged ventilation time (median 10 h vs 6 h, P = 0.001). Time in the ICU was longer in patients with 2 or more risk factors (median 9 days vs 4.5 days, P = 0.003). CONCLUSIONS: RV end-diastolic volume index >200 ml/m2, RV-EF <40% and age >50 years are helpful factors to identify patients with an increased perioperative risk. The results also suggest that earlier surgery may yield more favourable results in patients with Ebstein's anomaly.

Risk Factors for Failure of Systemic-to-Pulmonary Artery Shunts in Biventricular Circulation.

Systemic-to-pulmonary artery shunt placement is an established palliative procedure for congenital heart disease, but it is associated with high morbidity and mortality. Data of all patients with biventricular circulation who underwent systemic-to-pulmonary artery shunt implantation between 2000 and 2016 were reviewed. Endpoints of the study were shunt failure and shunt-related mortality. Shunt failure was defined as any shunt dysfunction requiring intervention or reoperation. Shunt-related mortality was defined as death due to shunt dysfunction. A total of 217 shunts (central shunt, n = 131, Blalock-Taussig shunt, n = 86) were implanted in 178 patients. The median age of the patients was 98 days [1 day to 1.2 years]. Corrective surgery was performed at a median time of 0.6 years [3 months to 7 years] after shunt placement. Shunt failure was diagnosed in 21 patients (9.6%) at a median time of 14.6 days [0 days to 2 years]. Causes of shunt failure were stenosis (n = 11; 5%) and thrombosis (n = 10; 4.6%). The rate of freedom from shunt failure was 89.9 ± 2.6% at 1 year, the rate of shunt-related mortality was 3% (n = 5), and the rate of freedom from shunt-related mortality at 1 year was 97.5 ± 1%. Platelet transfusion was required in 43 patients (20%), all for postoperative thrombocytopenia. Perioperative platelet transfusion (p = 0.03) and shunt size of 3 mm (p = 0.03) were identified as risk factors for shunt failure. Shunt size of 3 mm was also identified as a risk factor for shunt-related mortality. The ideal shunt size in patients with biventricular circulation requiring a systemic-to-pulmonary artery shunt is 3.5 mm or larger. Platelet transfusion increases the risk of shunt failure and should be avoided. Type of shunt and diagnosis have no influence on morbidity or mortality after shunt placement.

Long-term outcome of preadolescents, adolescents, and adult patients undergoing total cavopulmonary connection.

OBJECTIVES: Patients with a single ventricle infrequently undergo total cavopulmonary connection as preadolescents, adolescents, or adults. The purpose of this study was to clarify the characteristics of this cohort and to analyze the factors influencing outcomes. METHODS: Between 1994 and 2015, 50 of 460 patients underwent total cavopulmonary connection as preadolescents, adolescents, or adults (group A). The patients' characteristics and operative results were compared with those of the remaining 410 patients aged less than 9 years who underwent total cavopulmonary connection (group B). Post-total cavopulmonary connection echocardiogram reports (n = 4862) were used to evaluate longitudinal ventricular function, and ejection fraction was characterized using nonlinear mixed-effects models and compared between groups. RESULTS: The median follow-up time was 10.3 (2.8-15.5) years. The differences between groups in 30-day mortality (P = .20), intensive care unit stay (P = .20), and incidence of prolonged effusion (P = .08) were not significant. The estimated survival at 15 years was lower in group A (86.5%) than in group B (94.0%, P = .04) patients. Long-term systemic ventricular ejection fraction, analyzed with linear mixed-effect models, was significantly reduced in group A than in group B patients (P < .001). At a median postoperative period of 8.4 (7.1-10.5) years, the peak oxygen uptake as measured by exercise capacity testing was lower in group A than in group B patients, respectively (22.3 ± 6.5 [n = 25] vs 30.6 ± 8.1 [n = 100] mL/kg/min, P < .001). CONCLUSIONS: The total cavopulmonary connection procedure was performed in preadolescent, adolescent, and adult patients with no significant difference in 30-day or hospital mortality compared with those in young children. However, long-term survival and ventricular performance were reduced in this older cohort.

Outcomes of a total cavopulmonary connection in patients with impaired ventricular function.

OBJECTIVES: Our aim was to evaluate outcomes following a total cavopulmonary connection (TCPC) in patients with preoperatively impaired ventricular function (VF). METHODS: Of 483 consecutive TCPC patients, 44 (9.1%) had impaired VF (ejection fraction <50%, Group A), and 439 patients had normal VF (ejection fraction ≥50%, Group B). We compared the clinical outcomes between the groups. RESULTS: The median age at TCPC was 2.8 (interquartile range 1.9-8.3) years in Group A and 2.3 (1.8-3.5) years in Group B (P = 0.025). An atrioventricular valve (AVV) operation prior to (38.6 vs 27.1%, P < 0.001) and concomitant with (31.8 vs 12.1%, P < 0.001) the TCPC was performed more frequently in Group A. The median intensive care unit stay (7.0 vs 7.0 days, P = 0.737) and 30-day survival (97.7 vs 98.4%, P = 0.737) were not significantly different between groups. Freedom from death, transplantation (P = 0.115) and catheter intervention (P = 0.603) showed no difference between groups. However, freedom from cardiac reoperation was significantly lower in Group A (P < 0.001). VF was resolved in 22 of the 39 (56.4%) survivors in Group A. The recovered patients had a lower incidence of AVV reoperation (0 vs 6, P = 0.002) and pacemaker rhythm (0 vs 5, P = 0.006). CONCLUSIONS: In patients planned for TCPC, impaired VF is often associated with AVV regurgitation. TCPC can be performed with low risk and comparable clinical results except for cardiac reoperation in patients with impaired VF when compared to patients with normal VF. Following TCPC, VF recovers in half of the survivors. A competent AVV and sinus rhythm are prerequisites for recovery.

Long-term outcome after anomalous left coronary artery from the pulmonary artery repair: a 40-year single-centre experience.

OBJECTIVES: An anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, often associated with severely impaired left ventricular (LV) contractility and functional mitral valve (MV) regurgitation. Current data suggest that earlier correction of ALCAPA may result in a more complete recovery of LV function. By analysing the results of a large single-centre ALCAPA cohort, we sought to investigate whether these treatment paradigms remain valid. METHODS: A retrospective study was performed evaluating all patients undergoing repair of ALCAPA over a period of almost 40 years. All preoperative and postoperative echocardiographic reports were reviewed, focusing on the recovery of LV and MV function. RESULTS: The study cohort included 78 patients who underwent ALCAPA repair between 1977 and 2015, who were divided into 2 groups based on patient age at initial repair: Group A (n = 52, age <1 year) and Group B (n = 26, age >1 year). Following repair, systolic LV and MV function improved significantly (P < 0.01) in both groups. Patient age at the time of initial surgery had no significant influence on the improvement of LV function. Early mortality (within 30 days) was 10% (n = 8). No 30-day mortality was reported in the past 20 years. Survival at 20 years following ALCAPA repair was 86 ± 4%. CONCLUSIONS: Following ALCAPA repair, LV function significantly improved, regardless of age at the time of repair. In addition, preoperative functional MV regurgitation decreased over time. Concomitant mitral valve surgery at the time of ALCAPA repair is required in patients with structural abnormalities of the MV.

Atrioventricular valve regurgitation in patients undergoing total cavopulmonary connection: Impact of valve morphology and underlying mechanisms on survival and reintervention.

OBJECTIVE: The study objective was to determine the mechanisms of atrioventricular valve regurgitation in single-ventricle physiology and their influence on outcomes after total cavopulmonary connection. METHODS: Among 460 patients who underwent a total cavopulmonary connection, 101 (22%) had atrioventricular valve surgery before or coincident with total cavopulmonary connection. RESULTS: Atrioventricular valve morphology showed 2 separated in 33 patients, mitral in 11 patients, tricuspid in 41 patients, and common in 16 patients. Patients with a tricuspid and a common atrioventricular valve underwent atrioventricular valve surgery frequently, 27% and 36%, respectively. Atrioventricular valve regurgitation was due to 1 or more of the following mechanisms: dysplastic leaflet (62), prolapse (53), annular dilation (27), cleft (22), and chordal anomaly (14). Structural anomalies were observed in 89 patients (88%). The procedure was atrioventricular valve repair in 81 patients, atrioventricular valve closure in 16 patients, and atrioventricular valve replacement in 4 patients. Among 81 patients who underwent initial repair, repeat repair was required in 20 patients, atrioventricular valve replacement was required in 7 patients, and atrioventricular valve closure was required in 3 patients. Among patients undergoing atrioventricular valve surgery, overall survival after total cavopulmonary connection (88% vs 95% at 15 years, P = .01), freedom from atrioventricular valve reoperation after total cavopulmonary connection (75% vs 99% at 15 years, P < .01), and grade of atrioventricular valve regurgitation at a median follow-up of 6.6 years (P < .01) were worse than in those who did not require atrioventricular valve surgery. CONCLUSIONS: Atrioventricular valve regurgitation in univentricular heart is more frequently associated with a tricuspid or a common atrioventricular valve, and structural anomalies are the primary cause. Significant atrioventricular valve regurgitation requiring surgery influences survival after total cavopulmonary connection, especially when atrioventricular valve replacement was needed. Surgical management based on mechanisms of regurgitation is mandatory.

Impact of early Fontan completion on postoperative outcomes in patients with a functional single ventricle.

OBJECTIVES: Our aim was to evaluate whether early timing of total cavopulmonary connection (TCPC) affects postoperative outcomes. METHODS: Of 460 consecutive TCPC patients, 51 (11.1%) underwent TCPC ≤ 18 months of age (group A), and 409 patients >18 months of age (group B). We compared the clinical outcomes and exercise capacity between groups. RESULTS: Median age at TCPC was 1.4 (interquartile ranges: 1.3-1.5) years in group A and 2.5 (1.9-4.5) years in group B. Duration of intensive care unit stay (6 vs 7 days), hospital stay (20 vs 20 days), and 30-day survival (100 vs 98%) was not significantly different between groups. Estimated survival (95.3 vs 92.1%), freedom from reoperation (93.7 vs 86.3%), freedom from catheter intervention (60.1 vs 77.0%), and freedom from protein losing enteropathy (97.6 vs 93.8%) at 10 years was not significantly different between groups. At last follow-up, no patient in group A but 13 patients in group B exhibited reduced ventricular function ( P = 0.035). Exercise-capacity testing showed that both peak oxygen uptake (36.4 vs 28.6 ml/kg/min; P = 0.026) and its percentage of predicted value (82.9 vs 70.0%; P = 0.004) were significantly higher in group A ( n = 6, mean postoperative period: 8.9 years) than in group B ( n = 119, mean postoperative period: 8.7 years). CONCLUSIONS: Fontan completion for TCPC can be performed at an early age with a low perioperative risk and good intermediate results. Earlier unloading of the systemic ventricle and earlier elimination of chronic cyanosis by staged cavopulmonary connection might be advantageous for preservation of long-term ventricular function.

Does Platelet Reactivity Predict Bleeding in Patients Needing Urgent Coronary Artery Bypass Grafting During Dual Antiplatelet Therapy?

BACKGROUND: Up to 15% of patients require coronary artery bypass grafting (CABG) during dual antiplatelet therapy. Available evidence suggests an association between platelet reactivity and CABG-related bleeding. However, platelet reactivity cutoffs for bleeding remain elusive. We sought to explore the association between platelet reactivity and bleeding. METHODS: Patients on aspirin and a P2Y12 receptor inhibitor within 48 hours before isolated CABG (n = 149) were enrolled in this prospective study. Blood was drawn 2 to 4 hours preoperatively and platelet reactivity assessed by light transmittance aggregometry (LTA), vasodilator-stimulated phosphoprotein (VASP) assay, Multiplate analyzer and Innovance PFA2Y. The primary endpoint was calculated red blood cell loss computed as follows: (blood volume × preoperative hematocrit × 0.91) - (blood volume × hematocrit × 0.91 on postoperative day 5) + (mL of transfused red blood cells × 0.59). RESULTS: Preoperative platelet reactivity was low [median (interquartile range): LTA: 20 (9-28)%; VASP-PRI: 39 (15-73)%; Multiplate adenosine phosphate test: 16 (12-22) U∗min]. Innovance PFA2Y ≥300 seconds, 72%. Median (IQR) red blood cell loss in patients in first the LTA tertile was 1,449 (1,020 to 1,754) mL compared with 1,107 (858 to 1,512) mL and 1,075 (811 to 1,269) mL in those in the second and third tertiles, respectively (p < 0.004). Bleeding Academic Research Consortium (BARC)-4 bleeding differed between tertiles (62% versus 46% versus 36%; p = 0.037). In a multivariable linear regression model, aspirin dose ≥300 mg, cardiopulmonary bypass time, EuroSCORE, and tertile distribution of platelet reactivity were significantly associated with red blood cell loss. CONCLUSIONS: A gradual decrease in red blood cell loss and BARC-4 bleeding occurs with increasing platelet reactivity in patients on antiplatelet therapy undergoing CABG. Our findings support current guidelines to determine time of surgery based on an objective measurement of platelet function (Platelet Inhibition and Bleeding in Patients Undergoing Emergent Cardiac Surgery; clinicaltrials.gov NCT01468597).

Kawasaki disease and hepatobiliary involvement: report of two cases.

BACKGROUND: Kawasaki disease (KD) without affection of the coronary artery system is rare. Optic nerve pathology together with KD has not been described earlier. CASE PRESENTATION: We present one case of KD in a 12-year-old girl predominantly with prolonged cholestasis, and a second case of multiple recurrent KD in a 9-year-old boy with hepatomegaly and ischemic optic nerve neuropathy. The coronary artery system was not involved in either case. CONCLUSIONS: KD warrants rapid diagnosis and immediate specific treatment in order to prevent the high risk of coronary artery aneurysm and stenosis.

Effect of body position on ventilation distribution during PEEP titration in a porcine model of acute lung injury using advanced respiratory monitoring and electrical impedance tomography.

BACKGROUND: Lung failure after acute lung injury remains a challenge in different clinical settings. Various interventions for restoration of gas exchange have been investigated. Recruitment of collapsed alveoli by positive end expiratory pressure (PEEP) titration and optimization of ventilation-perfusion ratio by prone positioning have been extensively described in animal and clinical trials. This animal study was conducted to investigate the effects of PEEP and positioning by means of advanced respiratory monitoring including gas exchange, respiratory mechanics, volumetric capnography and electrical impedance tomography. METHODS: After induction of acute lung injury by oleic acid and lung lavage, 12 domestic pigs were studied in randomly assigned supine or prone position during a PEEP titration trial with maximal PEEP of 30 mbar. RESULTS: Induction of lung injury resulted in significant deterioration of oxygenation [partial pressure of arterial oxygen/inspiratory fraction of oxygen (PaO2/FiO2): p = 0.002] and ventilation [partial pressure of arterial carbon dioxide (PaCO2): p = 0.002] and elevated alveolar dead-space ratios (Valv/Vte: p = 0.003) in both groups. Differences in the prone and the supine group were significant for PaCO2 at incremental PEEP 10 and 20 and at decremental PEEP 20 (20d) and 10 (10d), for PaO2/FiO2 at PEEP 10 and 10d and for alveolar dead space at PEEP 10d. Electrical impedance tomography revealed homogenous ventilation distribution in prone position during PEEP 20, 30 and 20d. CONCLUSIONS: Prone position leads to improved oxygenation and ventilation parameters in a lung injury model. Respiratory monitoring with measurement of alveolar dead space and electrical impedance tomography may visualize optimized ventilation in a PEEP titration trial.

Re-sternotomy as an unwelcome side-effect of unknown effervescent tablet intake?

Four hours after surgery for aortic valve stenosis and tricuspid valve regurgitation, an unknown foreign body was present on the routine chest X-ray. We performed re-sternotomy in order to retrieve this foreign body. The foreign body was easy to move on fluoroscopy but we could not extract it. We concluded that the foreign body was in a subdiaphragmatic location. As a consequence, we performed gastroscopy. A white, frothy mass (similar to an undissolved effervescent tablet) within an ulcerated lesion was seen and partially extracted.

Systolic right ventricular function in children and young adults with pulmonary artery hypertension secondary to congenital heart disease and tetralogy of Fallot: tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging data.

OBJECTIVE: The tricuspid annular plane systolic excursion (TAPSE), as echocardiographic index to assess right ventricular (RV) systolic function, has not been investigated thoroughly in children and young adults with tetralogy of Fallot (TOF) and pulmonary artery hypertension secondary to congenital heart disease (PAH-CHD). PATIENTS: TAPSE values of 49 patients with PAH-CHD and 156 patients with TOF were compared with age-matched normal subjects. TAPSE values were also compared with RV ejection fraction (RVEF) and RV indexed end-diastolic volume (RVEDVi) determined by magnetic resonance imaging in PAH-CHD and TOF patients. RESULTS: Patients with a PAH-CHD showed a positive correlation between TAPSE with RVEF (r= 0.81; P < 0.001) and a negative correlation between TAPSE with RVEDVi (r=-0.67; P < 0.001). Similarly, in our TOF patients, a positive correlation between TAPSE with RVEF (r= 0.65; P < 0.001) and a negative correlation between TAPSE with RVEDVi (r=-0.42; P < 0.001) was seen. CONCLUSIONS: Significant pressure overload in PAH-CHD patients and volume overload in TOF patients lead to a decreased systolic RV function, determined by TAPSE and magnetic resonance imaging and to increased RVEDVi values, determined by MRI, with time.

Accurate preoperative echocardiography has more impact on prediction of long-term mortality than intra-operatively measured flow in coronary bypass grafts.

OBJECTIVE: Our study aimed to analyze the predictive value of intra-operative bypass graft flow measurements for long-term mortality. METHODS: A total of 1593 consecutive coronary artery bypass graft (CABG) patients routinely underwent intra-operative bypass graft flow measurements with the transit-time flow meter (TTFM: Cardiomed(®)). The results of the flow measurements and the demographics were analyzed retrospectively. RESULTS: The mean follow-up was 3.8 years (0.5-8.8 years) with no losses to follow-up. Overall mortality was 10.1%. The preoperative left ventricular ejection fraction (LVEF) (echocardiograph) was the highest independent predictor of long-term survival (hazard ratio 0.97, p = 0.004) in all groups. The univariate analysis for the CABG I group showed that besides LVEF, female gender (hazard ratio 3.6, p = 0.02) was also significant. For the CABG II group, additive EuroSCORE (European System for Cardiac Operative Risk) (ES) (hazard ratio 1.4, p = 0.0001) and age (hazard ratio 1.1, p = 0.001) were significant. In the CABG III group, ES (hazard ratio 1.2, p < 0.0001), age (hazard ratio 1.04, p = 0.001), IMA (hazard ratio 0.5, p < 0.0001) and concomitant aortic valve replacement (AVR) (hazard ratio 2.1, p = 0.03) were significant, in addition to the LVEF. CONCLUSION: With quality-controlled surgeons checked by intra-operative TTFM, accurate quantification of preoperative LVEF significantly predicts long-term outcome. Effective bypass graft flows failed to predict outcome in CABG patients, regardless of the degree of coronary artery disease (CAD) and concomitant AVR.

Primary cardiac undifferentiated sarcoma of the right atrium presenting with cardiac tamponade.

This report illustrates the serial imaging of a primary cardiac undifferentiated sarcoma of the right atrium using echocardiography, chest X-ray, and computed tomography. Transthoracic echocardiography on presentation showed an extensive mass of the right atrial free wall with an impending cardiac tamponade. Symptoms were controlled with pericardiocentesis, pericardial window, and radiotherapy but recurred 8 months later with pleural effusion and tumor spread to the great arteries. Primary cardiac sarcoma (PSC) is a rare and aggressive malignancy that is usually diagnosed late due to its nonspecific symptoms. Cytology and cardiac biopsy may be negative, and suspicion for the tumor is warranted in recurrent pericardial effusion. Analogous to parietal pleural biopsy in lung tumors with pleural effusion, parietal pericardial biopsy may be positive in PSC of the right atrium with pericardial effusion. Echocardiography is the major diagnostic tool and aids pericardiocentesis. Pericardial window may be useful for recurrent pericardial effusion but does not preclude its reaccumulation. There is no proven effective treatment for PSC, and treatments include surgical resection, cardiac transplant, chemotherapy, and radiotherapy. Despite its poor prognosis, symptomatic relief is important and attainable.

Bilateral phrenic nerve palsy following aortic valve surgery.

Bilateral phrenic nerve palsy is an extremely rare but serious complication of open cardiac surgery. We report the case of a 78-year-old female who underwent elective aortic valve replacement and coronary artery bypass grafting under hypothermic cardiopulmonary bypass. Despite otherwise unremarkable postoperative recovery, the patient could not be weaned off ventilatory support. A chest radiograph and radiological screening of the diaphragm confirmed the diagnosis of bilateral phrenic nerve palsy. Following bilateral hemi diaphragm placation and extensive respiratory rehabilitation the patient was discharged several months after initial surgery breathing independently. Literature review revealed only two similar cases. Many contributing factors have been described but the etiology of bilateral phrenic nerve palsy following open cardiac surgery still remains unclear. Raised awareness of this condition is essential.