Idiopathic enlargement of the right atrium masking left atrial aneurysm in a neonate.

An idiopathic enlargement of the right atrium is an extremely rare cardiac malformation. There are no established guidelines for the management of this disease, especially concerning medical versus surgical therapeutic approach and the timing for an operation. We report in this case about a neonate that first was treated conservatively until the age of 5 month and finally got an operative resection of the aneurysm. After surgery, unexpected complications occurred. A second aneurysm in the left atrium was demasked. Furthermore, a progressive dilatation of both atrial chambers after resection required regular follow-up and ongoing evaluation of treatment.

Dysregulated autophagy in restrictive cardiomyopathy due to Pro209Leu mutation in BAG3.

Myofibrillary myopathies (MFM) are hereditary myopathies histologically characterized by degeneration of myofibrils and aggregation of proteins in striated muscle. Cardiomyopathy is common in MFM but the pathophysiological mechanisms are not well understood. The BAG3-Pro209Leu mutation is associated with early onset MFM and severe restrictive cardiomyopathy (RCM), often necessitating heart transplantation during childhood. We report on a young male patient with a BAG3-Pro209Leu mutation who underwent heart transplantation at eight years of age. Detailed morphological analyses of the explanted heart tissue showed intracytoplasmic inclusions, aggregation of BAG3 and desmin, disintegration of myofibers and Z-disk alterations. The presence of undegraded autophagosomes, seen by electron microscopy, as well as increased levels of p62, LC3-I and WIPI1, detected by immunohistochemistry and western blot analyses, indicated a dysregulation of autophagy. Parkin and PINK1, proteins involved in mitophagy, were slightly increased whereas mitochondrial OXPHOS activities were not altered. These findings indicate that altered autophagy plays a role in the pathogenesis and rapid progression of RCM in MFM caused by the BAG3-Pro209Leu mutation, which could have implications for future therapeutic strategies.

The fate of the pulmonary autograft in the aortic position: experience and results of 98 patients in twelve years.

OBJECTIVE: The ideal prosthesis for aortic valve replacement in infants and adolescents is still controversially discussed. Implantation of mechanical prostheses or homografts is associated with serious risks such as reoperations, thromboembolic events and infections. This has led many surgeons to prefer the Ross operation. METHODS: Between 1996 and 2008 we performed Ross operations in 98 children and infants with a mean age of 11 years (range 1 mo-25 y), including concomitant procedures (n = 33). The operation was performed as a full-root replacement in 97 and as a subcoronary implantation in one patient. The patients were followed for up to twelve years (follow-up complete) to determine clinical and echocardiographic parameters. Mean duration of follow-up was 60 +/- 37 (range 3-150 months). RESULTS: Overall survival was 98 % (n = 96). In 70 patients (71.4 %) no autograft insufficiency (AI) could be observed. AI grade I was present in 22 patients (22.4 %), AI grade II in 3 patients (3 %), and AI grade III in one patient. The autograft in the latter patient was replaced by a mechanical prosthesis. Aortic root dilatation was observed in 10 patients (10.2 %). Z-score (median) in these patients was 4.1 (normal z-score < or = 2.4). One patient with aortic root dilatation was reoperated. A pacemaker was implanted in two patients. No patient is on constant anticoagulants. CONCLUSION: The anatomy and physiology of the LVOT is best restored by the implantation of a pulmonary autograft. This additionally provides a growth potential for infants at low risk for reoperations. Autograft dilatation is a risk that requires constant evaluation.

Factor XIII prevents development of myocardial edema in children undergoing surgery for congenital heart disease.

In a prospective investigation of perioperative cardiac edema formation requiring a delayed sternal closure, we identified thrombin increase combined with a simultaneous decrease of factor XIII as a probable cause. After experimental studies additionally revealed that factor XIII could protect endothelial barrier function, we did another prospective randomized trial in which factor XIII or placebo was preoperatively substituted. The substitution finally showed distinct effects minimizing the incidence of myocardial swelling. Therefore, the clinical application of factor XIII may have a valuable therapeutic benefit in cases of leakage syndrome during extracorporeal circulation in congenital heart surgery.

Superficial femoral vein in arterial reconstruction for limb salvage: outcome and fate of venous circulation.

BACKGROUND: In some particular cases in vascular surgery it is mandatory to perform arterial reconstruction using autologous graft. Since 1985, we have been using the superficial femoral vein for arterial substitute in the case of limb salvage, when another autologous vein has not been available. We made a prospective investigation on 32 patients over a mean period of 24.5+/-12.1 months (1 to 48 months) to evaluate the usefulness of superficial femoral vein (SFV) for distal arterial reconstructive surgery and to objectify the fate of venous circulation of the limb after removal of this vein. METHODS: There were 20 male and 12 female patients averaging 64.3+/-10.3 years, who underwent crural arterial reconstructive surgery using the composite technique (PTFE and SFV). All grafts had been placed subcutaneously in lateral to knee position. Mean length of removed superficial femoral vein was 13.2+/-9.4 cm. RESULTS: In 6 patients (18.7%), we found an early occlusion of bypass followed by major amputation in 5 cases (15.6%). Minor amputation had to be done in 12 patients (37.5%). Cumulative patency was 56.3% after 48 months. Following the removal of superficial femoral vein, we had no complications due to venous stasis. We found a significant increase of plethysmographically measured venous capacity (1.7+/-0.49 to 2.51+/-0.71 [p<0.01]) and venous outflow (14.9+/-5.34 to 23.9+/-10.4 [p<0.05]) after 7.7 months. We did not observe more significant changes of venous circulation during further follow-up. CONCLUSIONS: Despite overall good results, we only recommend the use of superficial femoral vein in hazardous situations, when other autologous material is not available.

Efficacy of inhaled iloprost in the management of pulmonary hypertension after cardiopulmonary bypass in infants undergoing congenital heart surgery. A case series of 31 patients.

INTRODUCTION: Reactive pulmonary hypertension is frequent in children with high pulmonary flow and pressure. Inhaled iloprost and nitric oxide are the only substances approved as selective pulmonary vasodilators, but data about the effectiveness and safety of inhaled iloprost during cardiac surgery in infants and children are limited. METHODS: We retrospectively analysed the effects of inhaled iloprost after cardiopulmonary bypass weaning on the ratio of mean pulmonary artery to mean arterial pressure. The effectiveness of the inhalation set up was tested in an in vitro study. RESULTS: Thirty-one patients received inhaled iloprost during surgery. The clinically used inhalation set up for inhaled iloprost delivered 20% to 30% (500 to 750 ng * kg-1) of the nebulizer dose and caused a decrease in the ratio of mean pulmonary artery to mean arterial pressure from 0.6±0.2 to 0.4±0.1 and 0.4±0.1 (30 and 60 minutes after)p <0.05. In eleven (35%) patients norepinephrine infusion was started. CONCLUSION: Our data suggest that a single dose of inhaled iloprost significantly decreases the ratio of mean pulmonary artery to mean arterial pressure for at least 60 min. Vasopressor support may be indicated to avoid systemic hypotension. The filled dose in the nebulizer should be high enough to compensate for the high depletion rate of the pediatric inhalation system. However, our study allows no final decision about beneficial or detrimental effects of the off label use of inhaled iloprost to reduce pulmonary artery pressure during congenital heart surgery.

Perioperative management in pediatric heart transplantation.

Peri- and early postoperative mortality significantly influences the probability of survival following heart transplantation in children. Main causes of death early after transplantation are rejection, non specific graft failure and RV failure due to pulmonary hypertension. Optimal therapy of pulmonary hypertension and aggressive use of assist devices as a bridge to recovery will substantially improve survival in the early period after transplantation. Furthermore, the use of marginal donor organs will be more acceptable because transient myocardial insufficiency may recover during extracorporeal life support.

Transvesical placement of one limb of an aortobifemoral bypass graft as a complication of aortic bypass surgery.

We report about a rare complication after aortobifemoral bypass surgery. In the present case the left limb of an aortobifemoral bypass graft was placed straight through the cavity of the urinary bladder. The diagnosis was found and ascertained by colour duplex ultrasound examination, cystoskopy and angiography. In consequence, we successfully performed open bladder surgery to replace the misplaced graft.

[Reopening of a persistent left superior vena cava in the early postoperative period following bidirectional cavopulmonary anastomosis--treatment by coil embolization]. / Frühpostoperative Eröffnung einer linkspersistierenden oberen Hohlvene nach bidirektionaler cavopulmonaler Konnektion - Coilembolisation als Therapie der Wahl.

Diagnosis of systemic venous drainage is mandatory for patients with congenital heart disease planned for cavopulmonary anastomosis or any Fontan-type palliation. Preexisting venous channels as the persistent left superior vena cava are common in cardiac anomalies and may lead to postoperative deterioration and cyanosis. We describe a 6 month old boy with a complex imbalanced atrioventricular septal defect who developed cyanosis in the very early postoperative period, following bidirectional cavopulmonary anastomosis. It was caused by ineffective lung perfusion due to a reopened persistent left superior vena cava with drainage to the coronary sinus.He underwent coil embolization of the persistent left superior vena cava with retrievable coils and cyanosis improved. Coil embolization is an effective alternative to secondary surgery, especially for hemodynamically compromised patients in the postoperative period.

Effect of intraoperative angiotensin-converting enzyme inhibition by quinaprilat on hypertension after coronary artery surgery.

Activation of the renin-angiotensin system during cardiopulmonary bypass (CPB) may be involved in early postoperative hypertension after coronary artery bypass grafting (CABG). As hypertensive episodes may be deleterious in the immediate postoperative period, we have assessed the effects of prophylactic treatment with the angiotensin-converting enzyme inhibitor quinaprilat in an open study. During steady state CPB, patients received quinaprilat 0.02 mg kg-1 (group A, n = 10), quinaprilat 0.04 mg kg-1 (group B, n = 10) or saline solution (group C, n = 10) as an i.v. bolus dose. Sodium nitroprusside (SNP) was given after operation when systolic arterial pressure was > 150 mm Hg. Requirements for SNP 1 h after arrival in the ICU were significantly less in groups A (two of 10) and B (two of 10) than in group C (eight of 10). Also, patients in group C had a greater systolic arterial pressure compared with groups A and B. There were no significant differences between groups in diastolic arterial pressure, heart rate, cardiac index or cardiac filling pressures. We conclude that quinaprilat can be used during CABG to reduce the incidence of postoperative hypertension. Further studies of the efficacy and safety of this technique are necessary.

Mycotic pseudoaneurysm of the aorta in children.

Mycotic pseudoaneurysm of the aorta is a rare disease in childhood. We report on two cases which were diagnosed in an unselected general pediatric population within an 8-month period. The first case was a 16-month-old toddler with a normal cardiac history who presented with purulent pericarditis due to group A streptococcus and subsequent pseudoaneurysm formation of the ascending aorta while convalescing from varicella infection. The second case was a 14-year-old girl with a previously undiagnosed coarctation of the aorta who developed a Staphylococcus aureus aortitis in the dilated poststenotic segment with pseudoaneurysm formation and infiltration into the adjacent lung tissue. In both cases parenteral antibiotic therapy was administered over 10 and 4 days, respectively, followed by emergency surgery consisting of aneurysmectomy, coarctectomy (case 2), and in situ homograft implantation. Recovery was uneventful. In both cases early institution of a femorofemoral cardiopulmonary bypass prevented a fatal outcome despite intraoperative rupture of the pseudoaneurysm.

The effect of a pre-operative infusion of Ringer's solution on splanchnic perfusion in patients undergoing coronary artery bypass grafting.

Surgical patients develop a fluid deficit during pre-operative starvation. This study examines the effects of pre-operative fluid administration on haemodynamic variables, oxygenation and splanchnic perfusion in patients undergoing elective coronary artery bypass grafting. Forty-eight patients were randomised to receive either a pre-operative crystalloid infusion (crystalloid group, n = 24) or no infusion (control group, n = 24). Patients in the crystalloid group received a continuous infusion of Ringer's solution at 1.5 ml.kg(-1).h(-1) from 22:00 h until induction of anaesthesia the next morning. Immediately before induction of anaesthesia, all patients were given a colloid infusion to increase pulmonary capillary wedge pressure and central venous pressure to similar levels in both groups. Haemodynamic and oxygenation parameters were measured using invasive cardiovascular monitoring, and splanchnic perfusion was assessed by indocyanine green clearance. Patients in the crystalloid group received a mean (SD) of 1008 (140) ml of Ringer's solution overnight. Patients in the crystalloid group had a higher splanchnic blood flow than the control group before induction of anaesthesia [mean (SD) = 1782 (573) ml.min(-1) vs. 1391 (333) ml.min(-1), p < 0.05]. There were no significant differences in systemic haemodynamic data and global oxygenation parameters between the two groups. Pre-operative infusion of crystalloid appears to result in an improvement in pre-operative splanchnic perfusion.

Incidence and risk calculation of inotropic support in patients undergoing cardiac surgery with cardiopulmonary bypass using an automated anaesthesia record-keeping system.

BACKGROUND: This retrospective study analysed the effects of preoperative and intraoperative factors on the occurrence of inotropic support after cardiopulmonary bypass (CPB). METHODS: The data sets of 1471 adult patients having received elective cardiac surgery with CPB were recorded using an online anaesthesia record-keeping system. Patients were judged to have required inotropic drug support if they had received one or a combination of the positive inotropic drugs, epinephrine, dobutamine and enoximone. The effects of age, height, weight, body mass index, gender, chronic heart failure, documented preoperative myocardial infarction, left main coronary artery disease, preoperative history of hypertension, chronic renal failure, diabetes mellitus, chronic obstructive pulmonary disease (COPD), preoperative medical treatment, type of surgical procedure, duration of CPB, duration of aortic clamping and reperfusion time were analysed by logistic regression for predictive power of the need for positive inotropic drugs. RESULTS: Of the patients, 32.4% received positive inotropic drugs in the operating theatre after weaning from CPB. The overall 30-day mortality was 2.2%. Of non-survivors, 81.8% received inotropes compared with 18.2% of survivors (P < 0.01). The numbers of previous myocardial infarctions (odds ratio (OR), 2.01), congestive heart failures New York Heart Association class > 2 (OR, 1.85), COPD (OR, 1.85) and age > 65 yr (OR, 1.62), aortic cross clamping time of > 90 min (OR, 2.32) and coronary artery bypass surgery (OR, 0.43) all represented influential factors within the logistic regression model. CONCLUSION: The knowledge of these risk factors should be useful in increasing the anaesthetist's vigilance in those patients most at risk for inotropic support and in providing for more timely therapeutic intervention and optimizing anaesthesia management.

[Cardiac troponin I after heart surgery corrective operation in infancy and childhood]. / Kardiales Troponin I nach kardiochirurgischer Korrekturoperation im Säuglings- und Kindesalter.

BACKGROUND: Perioperative myocardial damage is an important determinant for postoperative cardiac function and recovery. Cardiac troponin I (cTNI) is a specific marker for myocardial damage. The aim of our study was to evaluate pre- and postoperative cTNI levels, the pattern of elevation in the first four postoperative days and the prognostic value after pediatric cardiac operation. METHODS: Cardiac troponin I levels were measured in 115 children mean age 36 +/- 45 months (range 4 days to 189 months) undergoing elective operation of a congenital heart defect. Routine measurements were made preoperatively, immediately after cardiopulmonary bypass and serially 8, 18, 42, 90, 138 hours thereafter. Data from 13 patients undergoing surgery without cardiopulmonary bypass served as controls. Postoperative cTNI levels were correlated with intra- and postoperative parameters (such as duration of aortic crossclamping, cardiopulmonary bypass time and need for postoperative inotropic support). RESULTS: All preoperative cTNI levels were in the normal range. Postoperatively, the highest median cTNI levels were found in patients after repair of tetralogy of Fallot (TOF), atrioventricular septal defect (AVSD) and implantation of a homo- or xenograft. Postoperative cTNI levels correlated significantly with duration of cardiopulmonary bypass and aortic crossclamping, operative approach (ventriculotomy versus atriotomy) and inotropic support (p < 0.0001). Peak cTNI levels were found immediately after surgery in 77.4% of our patients, 8 hours postoperative in 13.9% and at 18 hours after the surgery in 5.2% of the patients. In three children cTNI continued to increase; a secondary increase was found in one patient. Two of these children died, two had a prolonged postoperative recovery. CONCLUSION: The postoperative level of cardiac troponin I could be used as a marker of perioperative myocardial injury caused by ischemia and operative trauma. Peak levels usually could be obtained immediately after surgery, but a further increase of cTNI during the following 18 hours may occur and is not necessarily related to impaired recovery. However still increasing cTNI levels after 18 hours postoperatively and a secondary increase as well may be used as indicators of poor outcome.

An assessment of selective cerebral perfusion via the innominate artery in aortic arch replacement.

Maintaining an adequate cerebral oxygen supply is a serious problem in aortic arch surgery. Deep hypothermic circulatory arrest is the most common method used for cerebral protection, but guarantees only a time-limited safety period. Based on experimental investigations, we applied selective cerebral perfusion via the innominate artery alone with only moderate hypothermia (28 degrees C) and without circulatory arrest in 25 consecutive patients undergoing surgical treatment of an aneurysm (n = 10) or acute type-A dissection (n = 15) involving the aortic valve and arch. In every case a test perfusion was carried out to assess whether the cerebral perfusion achieved would be adequate for the whole operation. In no case was the perfusion inadequate. As a new perioperative monitoring system, we used computer-aided topographical electroencephalometry (CATEEM). There were 18 male and 7 female patients, their age was 47.0 +/- 15.1 years (mean +/- SD). Mean time periods were 155.1 +/- 37.3 min for aortic cross-clamping, and 69.3 +/- 35 min for selective cerebral perfusion. Postoperatively, two patients (8%) revealed a temporary left-sided hemiparesis, and 4 patients (16%) died within 30 days. The overall mortality rate was 16% in a follow-up period of 24.2 +/- 9.5 months. In this small group the CATEEM monitoring enabled an intraoperative selection of patients with sufficient bihemispheric collateral circulation and therefore suitable for simple innominate artery perfusion.

Modification of surgical aortoatrial shunts for inaccessible bleeding in aortic surgery -- modification of the Cabrol-shunt technique.

Bleeding after complex ascending aortic, aortic root or transverse arch surgery which is inaccessible or difficult to control may present a major problem. Here, we describe a modified Cabrol-shunt technique using complete mediastinal coverage with decompression into the innominate vein where the classical technique is not suitable. The long-term fate of the classical aortoatrial and modified mediastinal to innominate shunts has been analyzed to assess their potential complications.

Heart transplantation in children and infants: short-term outcome and long-term follow-up.

Since 1988, 82 heart transplants have been performed in 80 infants and children. Diagnoses pretransplant were: hypoplastic left heart syndrome (HLHS) (n = 43); cardiomyopathy (n = 19); endocardial fibroelastosis (n = 6); and other complex congenital heart diseases (n = 12). Age at transplantation was < 1 yr in 61 patients. Overall survival rate was 79% at 1 yr and 73% at 5 and 10 yr. To date, 20 patients have died after transplantation. Causes of death were: rejection (eight patients); right ventricular failure (four patients); transplant coronary artery disease (TCAD) (two patients); and other causes (six patients). In the majority of patients somatic growth is not impaired, and renal function is reduced (but stable) in all patients. Two patients developed post-transplant lymphoproliferative disease, which was treated successfully. Major long-term morbidity is neurologic deficit - severe in three patients and minor in six. TCAD was present or suspected in six surviving patients. We conclude that heart transplantation in infants and children can be performed with good early and late results. Quality of life is excellent in most patients. TCAD, however, will become an increasing problem in the long term.

Intracellular adhesion molecule-1 in patients developing pulmonary insufficiency after cardiopulmonary bypass.

OBJECTIVES: Cardiopulmonary bypass activates adhesion molecules, which are associated with systemic inflammation and organ dysfunction. The intracellular adhesion molecule-1 (ICAM-1) has been evaluated in patients presenting pulmonary dysfunction after cardiac surgery. MATERIALS AND METHODS: Postoperative serum levels of the ICAM-1 were measured in 40 patients who underwent isolated coronary artery bypass grafting, in 28 with uneventful postoperative recovery (70 %) (Group 1), and in 12 (30 %) with postoperative respiratory insufficiency (Group 2), defined by the need for prolonged (> 24 hours) mechanical ventilation using a fractional oxygen concentration of > 40 %. RESULTS: Patients in group 1 were ventilated for 12.21 +/- 4.86 hours and those in group 2 for 92.91 +/- 48.14 hours (p < 0.001). ICAM-1 decreased from 145.98 +/- 73.40 ng/ml to 81.15 +/- 114.82 ng/ml in group 1, while in group 2 ICAM-1 showed a significant higher level and increased to 435.01 +/- 130.02 ng/ml (p < 0.001). The leukocyte count increased in both groups as well as the C-reactive protein (CRP) during the postoperative course. The CRP behaves similar in both groups (p = 0.636) in contrast to the leukocyte count which was significantly higher in group 2 (p < 0.01). While none of the patients in group 1 died the mortality in group 2 was 50 % (p < 0.001). CONCLUSION: Respiratory insufficiency after cardiopulmonary bypass is associated with a distinct increase in the ICAM-1. The reason for the increase of the ICAM-1 in this small subset of patients has not been clarified.