Haddad Syndrome

Congenital central hypoventilation syndrome (CCHS) causes predominantly sleep apnoea and is one of a growing number of inherited disorders characterised by autonomic nervous system dysfunction/dysregulation (ANSD). In association with Hirschsprung's disease (HSCR), it presents as Haddad's syndrome. We report a case of Haddad's syndrome complicated by sinus node dysfunction

Right ventricular myxoma in a patient with tetralogy of Fallot

INTRODUCTION: Cardiac myxoma is the most common primary cardiac tumour in adulthood and may present in the context of Carney's complex. PRESENTATION OF CASE: A 32-year-old male with a history of repaired tetralogy of Fallot in childhood was admitted with severe pulmonary valve regurgitation and a mobile mass in the right ventricle. The patient underwent pulmonary valve replacement and mass excision. Pathology examination showed myxoma. DISCUSSION: In the majority of cases myxomas originate in the atria, nevertheless they can also be found in a ventricular cavity. Myxoma is a prevalent feature of Carney's complex, an inherited, autosomal disease, characterised by multiple tumours in several organs. Tetralogy of Fallot has also been described in association with Carney's complex. CONCLUSION: Coexistence of tetralogy of Fallot with a cardiac ventricular myxoma in a patient not affected from Carney's complex or other familial syndrome

Postcardiotomy Mechanical Circulatory Support in Two Infants with Williams’ Syndrome

Supravalvar aortic stenosis (SVAS) in patients with Williams’ syndrome is often accompanied by coronary, pulmonary, and even myocardial lesions and therefore associated with increased perioperative morbidity and mortality. Extracorporeal membrane oxygenation (ECMO) provides reliable short-term mechanical circulatory support to patients, especially young, in acute postoperative cardiac failure when conventional means are ineffective. The incorporation of centrifugal pumps in these systems has made their use more efficient and less traumatic. We describe our experience of using the Levitronix CentriMag pump in two patients with Williams' syndrome who underwent surgical correction of supravalvular aortic stenosis

Pulmonary valve replacement in patients with corrected tetralogy of Fallot

Introduction: Development of pulmonary insufficiency in patients with surgically corrected tetralogy of Fallot (TOF) may lead to severe right heart failure with serious consequences. We herein present our experience with pulmonary valve replacement (PVR) in these patients. Methods: From 2005-2013, 99 consecutive patients (71 males/28 females, mean age 38±8 years), underwent PVR after 7 to 40 (mean 29 ± 8) years from the initial correction. Seventy nine of the symptomatic patients presented in NYHA II, 14 in III and 2 in IV. All underwent PVR with a stented bioprosthetic valve, employing a beating heart technique with normothermic extracorporeal circulation support. Concomitant procedures included resection of aneurysmal outflow tract patches (n = 37), tricuspid valve annuloplasty (n = 36), augmentation of stenotic pulmonary arteries (n = 9), maze procedure (n = 2) and pulmonary artery stenting (n = 4). Results: There were 2 perioperative deaths (2%). One patient developed sternal dehiscence requiring rewiring. Median ICU and hospital stay was 1 and 7 days respectively. Postoperative echocardiography at 6 and 12 months showed excellent bioprosthetic valve performance, significant decrease in size of the right cardiac chambers and reduction of tricuspid regurgitation (TR) in the majority of the patients. At mean follow-up of 3.6 ± 2 years, all surviving patients remain in excellent clinical condition. Conclusion: Probability of reoperation for pulmonary insufficiency in patients with surgically corrected TOF increases with time and timely PVR by preventing the development of right heart failure is crucial for long-term survival. Current bioprosthetic valve technology in combination with the beating heart technique provides excellent immediate and short-term results. Further follow-up is necessary to evaluate long-term outcome

Pulmonary valve replacement in patients with corrected tetralogy of Fallot

Introduction: Development of pulmonary insufficiency in patients with surgically corrected tetralogy of Fallot (TOF) may lead to severe right heart failure with serious consequences. We herein present our experience with pulmonary valve replacement (PVR) in these patients. Methods: From 2005-2013, 99 consecutive patients (71 males/28 females, mean age 38 +/- 8 years), underwent PVR after 7 to 40 (mean 29 +/- 8) years from the initial correction. Seventy nine of the symptomatic patients presented in NYHA II, 14 in III and 2 in IV. All underwent PVR with a stented bioprosthetic valve, employing a beating heart technique with normothermic extracorporeal circulation support. Concomitant procedures included resection of aneurysmal outflow tract patches (n = 37), tricuspid valve annuloplasty (n = 36), augmentation of stenotic pulmonary arteries (n = 9), maze procedure (n = 2) and pulmonary artery stenting (n = 4). Results: There were 2 perioperative deaths (2%). One patient developed sternal dehiscence requiring rewiring. Median ICU and hospital stay was 1 and 7 days respectively. Postoperative echocardiography at 6 and 12 months showed excellent bioprosthetic valve performance, significant decrease in size of the right cardiac chambers and reduction of tricuspid regurgitation (TR) in the majority of the patients. At mean follow-up of 3.6 +/- 2 years, all surviving patients remain in excellent clinical condition. Conclusion: Probability of reoperation for pulmonary insufficiency in patients with surgically corrected TOF increases with time and timely PVR by preventing the development of right heart failure is crucial for long-term survival. Current bioprosthetic valve technology in combination with the beating heart technique provides excellent immediate and short-term results. Further follow-up is necessary to evaluate long-term outcome

The arterial switch operation in Europe for transposition of the great arteries: A multi-institutional study from the European Congenital Heart Surgeons Association

Objectives: This study analyzes the results of the arterial switch operation for transposition of the great arteries in member institutions of the European Congenital Heart Surgeons Association. Methods: The records of 613 patients who underwent primary arterial switch operations in each of 19 participating institutions in the period from January 1998 through December 2000 were reviewed retrospectively. Results: A ventricular septal defect was present in 186 (30%) patients. Coronary anatomy was type A in 69% of the patients, and aortic arch pathology was present in 20% of patients with ventricular septal defect. Rashkind septostomy was performed in 75% of the patients, and 69% received prostaglandin. There were 37 hospital deaths (operative mortality, 6%), 13 (3%) for patients with an intact ventricular septum and 24 (13%) for those with a ventricular septal defect (P < .001). In 36% delayed sternal closure was performed, 8% required peritoneal dialysis, and 2% required mechanical circulatory support. Median ventilation time was 58 hours, and intensive care and hospital stay were 6 and 14 days, respectively. Although of various preoperative risk factors the presence of a ventricular septal defect, arch pathology, and coronary anomalies were univariate predictors of operative mortality, only the presence of a ventricular septal defect approached statistical significance (P = .06) on multivariable analysis. Of various operative parameters, aortic crossclamp time and delayed sternal closure were also univariate predictors; however, only the latter was an independent statistically significant predictor of death. Conclusions: Results of the procedure in European centers are compatible with those in the literature. The presence of a ventricular septal defect is the clinically most important preoperative risk factor for operative death, approaching statistical significance on multivariable analysis

Epidemiology and outcomes of hospital-acquired bloodstream infections in intensive care unit patients: the EUROBACT-2 international cohort study

Purpose In the critically ill, hospital-acquired bloodstream infections (HA-BSI) are associated with significant mortality. Granular data are required for optimizing management, and developing guidelines and clinical trials. Methods We carried out a prospective international cohort study of adult patients (≥ 18 years of age) with HA-BSI treated in intensive care units (ICUs) between June 2019 and February 2021. Results 2600 patients from 333 ICUs in 52 countries were included. 78% HA-BSI were ICU-acquired. Median Sequential Organ Failure Assessment (SOFA) score was 8 [IQR 5; 11] at HA-BSI diagnosis. Most frequent sources of infection included pneumonia (26.7%) and intravascular catheters (26.4%). Most frequent pathogens were Gram-negative bacteria (59.0%), predominantly Klebsiella spp. (27.9%), Acinetobacter spp. (20.3%), Escherichia coli (15.8%), and Pseudomonas spp. (14.3%). Carbapenem resistance was present in 37.8%, 84.6%, 7.4%, and 33.2%, respectively. Difficult-to-treat resistance (DTR) was present in 23.5% and pan-drug resistance in 1.5%. Antimicrobial therapy was deemed adequate within 24 h for 51.5%. Antimicrobial resistance was associated with longer delays to adequate antimicrobial therapy. Source control was needed in 52.5% but not achieved in 18.2%. Mortality was 37.1%, and only 16.1% had been discharged alive from hospital by day-28. Conclusions HA-BSI was frequently caused by Gram-negative, carbapenem-resistant and DTR pathogens. Antimicrobial resistance led to delays in adequate antimicrobial therapy. Mortality was high, and at day-28 only a minority of the patients were discharged alive from the hospital. Prevention of antimicrobial resistance and focusing on adequate antimicrobial therapy and source control are important to optimize patient management and outcomes