Internal mammary artery grafting for coronary hypoperfusion in children.

BACKGROUND: Pediatric coronary artery bypass is performed for ischemic complications of Kawasaki disease, congenital coronary anomalies, and iatrogenic pediatric coronary artery problems. METHODS: We performed myocardial revascularization using the internal mammary artery in 6 children. A review of outcomes is presented here. Patient ages ranged from 7 days to 10 years. RESULTS: There was no operative mortality. All coronary bypass grafts were patent at 3 months to 3 years postoperatively. No patient had recurrent angina or showed signs of myocardial ischemia. CONCLUSION: Internal mammary artery bypass grafting can be successfully performed in infants and children with good results. Long-term patency and growth of the anastomosis of the distal vessel are not clear, but good clinical and angiographic results have been reported even after 25 years.

Moderate patient-prosthesis mismatch after valve replacement for severe aortic stenosis has no impact on short-term and long-term mortality.

BACKGROUND: The importance of moderate patient-prosthesis mismatch (PPM) for the prognosis of patients who undergo aortic valve replacement is unclear. METHODS: The presence of PPM was assessed in 361 consecutive patients undergoing valve replacement for isolated severe aortic stenosis and related to perioperative and postoperative mortality. Indexed effective orifice areas (EOAi) were estimated for each type and size of prosthesis. RESULTS: Using the previously proposed cut-off of EOAi

Can hypocapnia reduce cerebral embolization during cardiopulmonary bypass?

BACKGROUND: Cerebral embolization is a major cause of central nervous dysfunction after cardiopulmonary bypass. Experimental studies demonstrate that reductions in arterial carbon dioxide tension (PaCO2) can reduce cerebral embolization during cardiopulmonary bypass. This study examined the effects of brief PaCO2 manipulations on cerebral embolization in patients undergoing cardiac valve procedures. METHODS: Patients were prospectively randomized to either hypocapnia (PaCO2 = 30 to 32 mm Hg, n = 30) or normocapnia (PaCO2 = 40 to 42 mm Hg, n = 31) before aortic cross-clamp removal. With removal of the aortic cross-clamp embolic signals were recorded by transcranial Doppler ultrasonography for the next 15 minutes. RESULTS: Despite significant differences in PaCO2, groups did not differ statistically in total cerebral emboli counts. The mean number of embolic events was 107 +/- 100 (median, 80) in the hypocapnic group and 135 +/- 115 (median, 96) in the normocapnic group, respectively (p = 0.315). CONCLUSIONS: Due to the high between-patient variability in embolization, reductions in PaCO2 did not result in a statistically significant decrease in cerebral emboli. In contrast to experimental studies, the beneficial effect of hypocapnia on cerebral embolization could not be demonstrated in humans.

Growth of the pulmonary autograft after the Ross operation in childhood.

OBJECTIVE: Excellent hemodynamic performance has been demonstrated after aortic valve replacement using the autologous pulmonary valve as described by D. Ross. However, in the pediatric population there is concern in regard to growth of the autograft and late dilatation in the systemic circulation. METHODS: Since 1991, 30 children (mean age, 11.3+/-3.1 years) had aortic valve replacement with the pulmonary autograft as a root replacement. All children had yearly clinical and echocardiographic follow-up. RESULTS: There were no perioperative deaths; one child died late in a car accident. At the last follow-up (mean follow-up, 4.3+/-2.6 years), all patients were in NYHA class I. There was one early reoperation, in which the autograft had to be reconstructed due to a leaflet perforation. There were no major valve related events. All children showed normal somatic growth. The annulus diameter increased significantly from 18+/-2 at surgery to 20+/-3.5 mm at the latest follow-up (P<0.004). The sinus also increased significantly in diameter from 29+/-4 at surgery to 34+/-2 mm at the last follow-up (P<0.001). This increase in autograft size, both for the annulus and the sinus, paralleled the increase in body surface area with no evidence for unproportional dilatation. Hemodynamic measurements demonstrated physiological peak gradients of 6.8+/-2.9 mmHg and no or trivial aortic insufficiency in 95% of this rapidly growing patient population. CONCLUSION: These data demonstrate growth of the pulmonary autograft parallel to somatic growth without undue dilatation in the systemic circulation. The hemodynamics are excellent with regard to physiological gradients and no increase in aortic insufficiency.

Neonatal and pediatric extracorporeal membrane oxygenation using nonocclusive blood pumps: the Vienna experience.

Neonatal and pediatric extracorporeal membrane oxygenation (ECMO) is carried out commonly using occlusive blood pumps. Centrifugal pumps provide simple and safe technology for transportation on ECMO. The assistence respiratoire extra corporelle (AREC) system enables single needle venovenous ECMO for infants. We report on our experience with neonatal and pediatric ECMO treatments using nonocclusive blood pumps. One-hundred forty-six ECMO treatments were performed for cardiac, neonatal, and pediatric indications in 54, 19, and 27% of cases. Centrifugal pumps were used in 99, and the AREC system in 42 cases. Hospital mortality was estimated retrospectively and influence of type of pump, type of ECMO belonging to indication group, and lactate at ECMO installation were estimated. Irreversible organ failure leading to ECMO termination was investigated within groups of indications. Survival (recent 50 ECMO treatments) was 80, 70, 43, and 30% after meconium aspiration syndrome, acute respiratory distress syndrome, cardiac surgery, and prolonged resuscitation. Lactate exceeding 100 mg/dl at ECMO installation predicted significantly worse outcome. Cerebral damage was the main reason for ECMO termination in all but persistent circulatory failure in the cardiac group. Myocardial recovery resulted in all except 2 cardiac cases. Nonocclusive blood pumps can be used safely in neonatal and pediatric ECMO. Early installation may improve outcome markedly. In cardiac cases results of surgery should be thoroughly investigated on the table before ECMO installation to prevent hopeless ECMO treatments.

[Partial left ventriculectomy (Batista-procedure) as an alternative to transplantation and as a rescue procedure]. / Die partielle linke Ventrikulektomie (Batista-Operation) als Alternative zur Transplantation und als chirurgische Notfallmassnahme.

Partial left ventriculectomy (Batista operation) is one of several surgical options for the treatment of end-stage heart disease. In a 17-year-old patient who could not be accepted as a candidate for heart transplantation, this procedure was performed as an acute rescue procedure in conjunction with reduction of the left ventricle, single-stitch reconstruction of the mitral valve and removal of a ventricular thrombus. Following temporary dependence on mechanical circulatory support the patient was transferred to his own country. The clinical experience is discussed, including aspects of the surgical technique, postoperative complications, indication, relevance of mitral reconstruction and rhythm problems. It is concluded that partial left ventriculectomy can be used to treat end-stage dilated cardiomyopathy, even as an emergency operation. Further studies and experience are needed to clarify the long-term effects and clinical limitations of the procedure.

Surgical aspects of pediatric cardiac extracorporeal membrane oxygenation.

Because children with severe myocardial dysfunction have limited therapeutic options, mechanical support of a failing heart is a matter of great interest. In the setting of cardiogenic shock or severe low cardiac output and hypoperfusion, extracorporeal membrane oxygenation (ECMO) can produce decisive improvements. The criteria for successful treatment include appropriate patient selection, improved surgical techniques and experience, higher recognition and anticipation of complications, and minimized delay in initiation of ECMO. Because the need for mechanical circulatory support may arise pre-, intra-, and postoperatively, every pediatric cardiac surgeon must be familiar with the principles and the surgical aspects of ECMO.

Proposed entry criteria for postoperative cardiac extracorporeal membrane oxygenation after pediatric open heart surgery.

While extracorporeal membrane oxygenation (ECMO) is being used increasingly after pediatric cardiac surgery, criteria are lacking for initiating ECMO after bypass weaning. To develop clinically useful ECMO entry criteria based on parameters readily available, children were examined at postoperative pediatric intensive care unit (PICU) admission. Using hospital mortality as the primary outcome, univariate and multiple logistic regressions were performed to estimate the predictive value of clinical (age, weight, and diagnosis) and laboratory (arterial blood pressure, pH, lactate, creatine kinase, and arterial and central venous oxygen saturation [ScvO2]) variables. Data from 218 children over a 2 year period were analyzed retrospectively. Univariate regression demonstrated that age, weight, diagnosis, blood pressure, venous and arterial saturation, and lactate were significantly associated with postoperative mortality (p < 0.05). In multiple regression, ScvO2 and lactate level were found to be independent predictors and were used in a predictive model (ScvO2 odds ratio: 2.03-828.6, p = 0.016) (lactate odds ratio: 1.58 -4.20, p = 0.0002) (R2 = 0.70). Applying an 80% risk of mortality to establish entry criteria as in neonatal ECMO, PICU admission values of lactate > 70 mg/dl if ScvO2 < 60% or lactate >163 mg/dl if ScvO2 > 60% are proposed to serve as postoperative ECMO entry criteria if bypass weaning has been possible but is followed by low cardiac output.

Acute clinical deterioration in a patient with end-stage dilated cardiomyopathy and left ventricular thrombus formation.

The case of a 17-year-old male patient with severe end-stage dilated cardiomyopathy and a large thrombus formation within the cavum of the left ventricle is reported. After an acute thrombectomia combined with a partial left ventriculectomy (Batista procedure), the patient was successfully treated with an appropriate left ventricular assist device (LVAD) system using a centrifugal nonocclusive pump (Biomedicus, Medtronic, Anaheim, CA, U.S.A.). Mechanical support was removed on Day 9, and the patient was discharged from the hospital on Day 19. The effectiveness of emergency mechanical support in patients with very unfavorable prognoses is discussed.

Excessive bleeding on extracorporeal membrane oxygenation after surgical repair of type I truncus arteriosus: A case report.

Severe bleeding remains the most common complication of extracorporeal membrane oxygenation (ECMO) following surgical repair of congenital heart defects. We present a case of excessive hemorrhage within the first hours on ECMO support after repair of a type I truncus arteriosus. Bleeding control was achieved by surgical repair following failure of conventional interventions to control hemorrhage despite normalization of laboratory coagulation parameters. Aspects associated with bleeding and bleeding control during extracorporeal circulation after cardiac surgery are discussed.

Valve-preserving treatment of Ebstein's anomaly: perioperative and follow-up results.

BACKGROUND: Ebstein's anomaly is a rare congenital cardiac defect of the tricuspid valve (TV) leading to severe tricuspid insufficiency (TI). METHODS: In ten patients, 6 adults (39-53 years) and 4 children (5-10 years), operated between 1989 and 1995 echocardiography was performed pre and post repair and at follow-up. Patients were assessed in our institution at two cut-off points, resulting in a mean first follow-up of 17 +/-15 months and a mean second follow-up of 53+/-23 months. All patients had additional congenital cardiac defects (ASD,VSD). In all patients the TV was repaired by techniques described by Carpentier et al. with some modifications. The goal of this reparative attempt is to mobilize restricted leaflet tissue and aid coaptation through implantation of a ring. RESULTS: Echocardiographically we were able to identify significant characteristics for the successful repair of Ebstein's anomaly. The severity of the disease is represented by the size and function of the right ventricle and the atrialized chamber, the most advanced cases exhibiting a dilated right ventricle with poor contractility. There was severe preoperative TI ( mean grade 3.2 +/- 0.3). Postoperatively TI was significantly reduced to a mean grade of 2+/-0.2. 60% of the patients demonstrated an improvement in the ratio of atrialized chamber to functional right ventricle. Right-ventricular function was improved, the mean score being 2.8+/-0.1. At follow-up I and II right-ventricular function and tricuspid insufficiency was improved in most patients and all patients benefited in quality of life. CONCLUSION: These results suggest that surgical correction should not be delayed until severe right heart failure develops as, particularly in children, good results are achieved, improving the quality of life.

Mechanisms underlying aortic dilatation in congenital aortic valve malformation.

BACKGROUND: The high incidence of aortic disease in subjects with congenital aortic valve malformations suggests a causative relationship between these 2 conditions. The histological observation in aortic dilatation/aneurysm/dissection is Erdheim cystic medial necrosis (CMN), a noninflammatory loss of smooth muscle cells (SMCs), fragmentation of elastic fibers, and mucoid degeneration. METHODS AND RESULTS: To examine whether apoptosis is 1 of the mechanisms underlying CMN and aortic medial layer SMC loss, ascending aortic wall specimens from 32 patients were collected at cardiothoracic surgery and examined by histochemical staining and terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end labeling. From echocardiography results, 4 groups of patients were identified: bicuspid valve carriers with (bi/dil) or without (bi/0) aortic dilatation and tricuspid valve carriers with (tri/dil) or without (tri/0) aortic dilatation. Massive focal apoptosis was observed in the medial layers of bi/dil (mean apoptotic index [mAI], 8.1+/-6.0) and tri/dil (mAI, 8.1+/-8.3) compared with tri/0 (mAI, 0.9+/-1.2; P=0.0079 and P=0.037). In bi/0 (mAI, 9.1+/-5.7) compared with tri/0 (mAI, 0.9+/-1.2), rates of medial SMC apoptosis were increased (P=0.0025). Bi/dil (mean age, 40. 6+/-15.7 years) were significantly younger than tri/dil (mean age, 56.4+/-12.8 years) undergoing the same operation (P=0.0123). CONCLUSIONS: Premature medial layer SMC apoptosis could be part of a genetic program underlying aortic disease in patients with aortic valve malformations.

[Hypoplastic left-heart syndrome. Initial intensive care experiences with the Norwood operation in Vienna]. / Hypoplastisches Linksherz-Syndrom. Erste intensivmedizinische Erfahrungen mit der Norwood-Operation in Wien.

Palliative surgery of the hypoplastic left heart syndrome (HLHS), whereby both pulmonary and systemic circulation are restored, was first described by Norwood in 1983. Careful ventilatory and pharmacologic modulation of the ratio of pulmonary to systemic vascular resistance are a crucial part of pre-, peri- and postoperative management. We report our experience in 3 of 7 newborns with HLHS who underwent the Norwood operation. Hemodynamic and respiratory parameters were evaluated retrospectively in these patients and we analysed the influence of diagnostic and therapeutic interventions on the course of disease before and after operation. During prostaglandin therapy two of three patients required mechanical ventilation preoperatively because of pulmonary hyperperfusion. Decreased myocardial contractility, oliguria and increased pulmonary vascular resistance characterized the postoperative course. The management included a careful application of inotropic support when necessary, adaptation of the ventilatory setting in order to modulate pulmonary perfusion and, in addition, institution of peritoneal dialysis. One patient died from staphylococcus aureus and superinfection with respiratory syncytial virus on day 41 after the operation. Maintaining an optimal balance between pulmonary and systemic blood flow is an essential aspect of postoperative management. Serum lactate and central venous oxygen saturation are helpful parameters in monitoring therapeutic measures in these patients. We conclude from our preliminary experience, that the Norwood operation might be an alternative therapeutic approach for newborns with HLHS in whom heart transplantation is not possible.

IgG subclass reactivity to human cardiac myosin in cardiomyopathy patients is indicative of a Th1-like autoimmune disease.

Studies performed in mice together with the demonstration of increased levels of heart-specific autoantibodies, cytokines and cytokine receptors in sera from cardiomyopathy (CMP) patients argued for a pathogenic role of autoimmune mechanisms in CMP. This study was designed to analyse the presence of IgG anti-heart antibodies in sera from patients suffering from hypertrophic and dilatative forms of CMP as well as from patients with ischaemic heart disease and healthy individuals. Patients' sera were analysed for IgG reactivity to Western-blotted extracts prepared from human epithelial and endothelial cells, heart and skeletal muscle specimens as well as from Streptococcus pyogenes. The IgG subclass (IgG1-4) reactivity to purified human cardiac myosin was analysed by ELISA. While sera from CMP patients and healthy individuals displayed comparable IgG reactivity to a variety of human proteins, cardiac myosin represented the prominent antigen detected strongly and preferentially by sera from CMP patients. Pronounced IgG anti-cardiac myosin reactivity was frequently found in sera from patients with dilatative CMP and reduced ventricular function. ELISA analyses revealed a prominent IgG2/IgG3 anti-cardiac myosin reactivity in CMP sera, indicating a preferential Th1-like immune response. Elevated anti-cytomegalovirus, anti-enterovirus IgG titres as well as IgG reactivity to nitrocellulose-blotted S. pyogenes proteins were also frequently observed in the group of CMP patients. If further work can support the hypothesis that autoreactivity to cardiac myosin represents a pathogenic factor in CMP, specific immunomodulation of this Th1- towards a Th2-like immune response may represent a promising therapeutic strategy for CMP.

Evidence against the current hypothesis of "gene dosage effects" of trisomy 21: ets-2, encoded on chromosome 21" is not overexpressed in hearts of patients with Down Syndrome.

BACKGROUND: The major current concept for the pathogenesis of the Down Syndrome (DS) phenotype including congenital heart disease (CHD) is the so-called "gene dosage effect." According to this hypothesis, genes encoded by chromosome 21 at the "critical region" (which is thought to be crucial for the development of the DS phenotype) are overexpressed in the trisomic state, thus leading to an imbalance of genes as, e.g., the protooncogene ets-2, superoxide dismutase, etc. METHODS: We studied heart biopsies obtained at surgery from 6 patients with DS and 7 patients with congenital heart disease. ets-2-mRNA steady state levels were determined by a competitive reverse transcription-polymerase chain reaction (RT-PCR) technique which allowed the determination of this gene at the attomol level. RESULTS: ets-2 mRNA in total ventricular tissue of DS patients showed concentrations of 0.60 +/- 0.42 fg/10 ng total RNA (mean, +/- SD). When normalized versus the housekeeping gene beta-actin to rule out general transcriptional changes in that disorder, the ratio of 0.56 +/- 0.28 (mean, +/- SD) was calculated. ets-2 mRNA in total ventricular tissue of patients with non-DS CHD showed concentrations of 0.45 +/- 0.22 fg/10 ng total RNA (mean, +/-SD) and ratios of 0.48 +/- 0.35 (mean, +/-SD). No differences could be found at the p<0.05 level. CONCLUSION: No absolute quantification of a gene incriminated in the "gene dosage effect-hypothesis" was performed so far and the only approach to (semi-) quantitative determination of the ets-2 gene using northern blotting was published on one individual DS sample only. This is the first report to clearly show that no overexpression of ets-2 can be found in heart of patients with DS, thus providing evidence against the current gene dosage effect-hypothesis.

Reversal of ventricular dilatation after correction of aortic incompetence: mechanical prosthesis compared with biological procedures.

BACKGROUND: The aim of the study was to compare early differences in reversal of LV dilatation between patients with mechanical prosthesis = group A (n = 51: Carbomedics = 40, Tekna/Edwards = 11) and biological procedures = group B (n = 75: pulmonary autograft = 36, aortic valve repair = 29, homograft = 10). METHODS: Since 1,990,126 consecutive patients younger than 50 years who had surgical correction of isolated aortic incompetence underwent echocardiographic examinations preoperatively, at discharge, and at one-year follow-up. Left-ventricular (LV) diameters were measured (LVEDD, LVESD) and matched to body surface area (LVESDI, LVEDDI and fractional shortening (FS) was calculated. Aortic peak flow velocities were assessed by Doppler technique and gradients were calculated. RESULTS: There were no significant differences preoperatively in aortic incompetence, NYHA classification, LVEDDI, LVESDI, and FS. In group B there was a significant decrease of LVESDI (p < 0.002) and LVEDDI (p < 0.001) but no change in FS at discharge. In group A a significant reduction of FS (p < 0.05) without any significant changes in LV size was observed. No patient died perioperatively or during the first year. At one-year follow-up (complete in 97.6% patients) there were no significant differences in LV diameters but group B had better ventricular function (p < 0.05) resulting in better NYHA classification (p < 0.05). Only group B had normal aortic valve gradients at discharge and at follow-up (A: 25.2 +/- 4.3 vs B: 10.2 +/- 2.4 mmHg). CONCLUSIONS: Normal aortic valve gradients in patients after aortic valve repair or allograft replacement for chronic aortic incompetence lead to early recovery from ventricular dilatation and significantly better ventricular function at discharge. One year postoperatively they had improved ventricular function and NYHA class in comparison with patients in whom a mechanical prosthesis was implanted.

Outer radius-wall thickness ratio, a postmortem quantitative histology in human coronary arteries.

Although the anatomy, histology and pathology of human coronary arteries have been studied extensively, little is known about the functional relationship between vessel radius and wall thickness. It is the purpose of this study to present detailed measurements and to describe this relationship covering the range from the feeding coronary artery to the arterioles. Human hearts of 10 adults less than 36 +/- 3 years old were investigated immediately postmortem. Ten cubic tissue blocks, measuring about 10 mm in length on each side, were dissected from the left ventricular wall. After fixation by immersion, 15-microm sections were prepared and outer and inner perimeters of 52 arterial segments were digitalized. Vessel radius and wall thickness were calculated and plotted to show their relationship over the whole range of vessel calibers. Outer vessel radii ranged from 100 to 3,000 microm and wall thickness from 80 to 800 microm. Plotting the outer vessel radius against the wall thickness, the data points were found to cluster around a straight line. A significant correlation between the two parameters was found (R2 = 0.79). This mathematical correlation and the good agreement of the presented results with data from other species indicate a common physiologic concept.

Single needle venovenous extracorporeal membrane oxygenation using a nonocclusive roller pump for rescue in infants and children.

In 1993, J.Y. Chevalier described a single needle venovenous extracorporeal membrane oxygenation (ECMO) system using a nonocclusive roller pump and alternating clamps for pulmonary support in neonates. We modified this system to use it in older children as well and for additional indications. Introducing a double raceway and 2 different sizes of tubing sets and performing percutaneous approach, we treated 21 children (age 1 day to 49 months) using this system. Indications for treatment were hypoxia and hypoxic induced myocardial dysfunction resulting from pulmonary failure, sepsis, and congenital defects. Of the children treated for neonatal indications, 7/9 survived. For 2 children ECMO was terminated because of intraventricular hemorrhage (IVH). In the pediatric group 5/7 of the children could be weaned from ECMO, and 2 children died after more than 30 days on ECMO. Two of the children who had been almost completely weaned died later because of therapy withdrawal following a brain death diagnosis. In the cardiac group, 3/5 of the children survived. We conclude that the described system is an effective venovenous ECMO system that reduces invasivity and expenditure.