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Extracorporeal membrane oxygenation for severe heart failure after Fontan operation.

A 10-year-old boy with tricuspid atresia and Glenn anastomosis underwent a modified Fontan operation. After the operation, the pressure in the inferior vena cava increased, leading to oliguria and ascites. After the creation of continuity between the superior vena cava and the inferior vena cava to reduce the pressure gradient, there remained an elevated right atrial pressure. Six days of extracorporeal membrane oxygenation effectively stabilized his hemodynamics and organ function and allowed an excellent outcome.

Angiography↗

The electrophysiologic characteristics of atrioventricular nodal reentry tachycardia with eccentric retrograde activation.

BACKGROUND: The occurrence of eccentric retrograde atrial activation has been demonstrated to be from 6 to 8% in patients with atrioventricular nodal reentrant tachycardia (AVNRT) by several previous reports. However, most of those reports were limited by the absence of coronary sinus venography to confirm if the retrograde activation was truly left sided. The purposes of this study were to 1) determine the incidence of left sided retrograde atrial activation in our center, 2) determine the specific electrophysiologic characteristics of eccentric and concentric atrial activation and 3) determine the outcome of radiofrequency catheter ablation for AVNRT with eccentric retrograde atrial activation. METHODS: From November 2001 to July 2004, 290 consecutive patients with AVNRT who underwent an electrophysiologic study and radiofrequency ablation were included. Group 1 consisted of AVNRT patients with eccentric retrograde atrial activation; group 2 consisted of AVNRT patients with concentric retrograde atrial activation. The electrophysiologic characteristics of the group 1 and group 2 patients were then compared. RESULTS: The incidence of AVNRT with eccentric retrograde activation confirmed by CS venography was 6.5%. There were more females and atypical AVNRT in patients with retrograde eccentric conduction. There was more VA block after ablation and tachycardia induction by right ventricular pacing/extrastimuli in eccentric rather than concentric retrograde atrial activation. A shorter antegrade fast functional refractory period of the AV node was demonstrated in the atypical eccentric group as compared to the atypical concentric group. CONCLUSION: This study demonstrated the different electrophysiologic characteristics between the AVNRT patients with eccentric and concentric retrograde atrial activation. Successful ablation sites were similar to the standard RA ablation sites in patients with retrograde eccentric conduction.

Adolescent↗

Successful palliation of acute superior vena caval obstruction after the Senning operation.

Superior vena caval obstruction after an intraatrial baffle procedure can lead to acute cerebral complications and hence requires immediate management. We present a case of successful palliation of acute superior vena caval obstruction after a Senning procedure by establishing a venous shunt between the innominate vein and pulmonary artery. This technique resulted in immediate hemodynamic and functional improvement that subsequently allowed for the enlargement of the superior vena cava-right atrial junction with a pericardial patch.

Acute Disease↗

Bidirectional Glenn shunt in association with congenital heart repairs: the 1(1/2) ventricular repair.

BACKGROUND: The bidirectional Glenn shunt has been used to incorporate a smaller tripartite ventricle into the circulation and create pulsatile pulmonary artery flow. We reviewed our operative experience and assessed hemodynamics of the bidirectional Glenn shunt in 1(1/2) ventricular repair or in conjunction with other repairs of congenital heart defects. METHODS: Between 1992 and 1998, 15 patients (mean age, 8.1+/-7.9 years) had bidirectional Glenn shunt in association with repair of congenital heart defects. Eighty-seven percent had at least one previous operation. All patients had simultaneous or previous intracardiac repair and had bidirectional Glenn shunt to volume unload the small right ventricle (group A, n = 7), to unload the poorly functioning right ventricle (group B, n = 2), to redirect superior vena cava-pulmonary venous atrial connection to treat cyanosis (group C, n = 2), or to unload the pulmonary left ventricle for residual intracavitary hypertension in patients with L-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (group D, n = 4). Intraoperative hemodynamic assessment was done in 2 patients in group A by selective use of inflow occlusion and flow probes. RESULTS: All patients survived. Four patients had successful, concurrent arrhythmia circuit cryoablation for Wolf-Parkinson-White syndrome (n = 1) or atrial reentry tachycardia (n = 3). Superior and inferior vena caval flow averaged 36% and 64% of cardiac output, respectively. Postoperative superior vena caval pressure (n = 13) was 13.7+/-4.0 mm Hg with pulmonary arterial flow pattern contributed by the ventricle in systole (pulsatile) and the superior vena cava in diastole (laminar). CONCLUSIONS: The bidirectional Glenn shunt is an effective adjunct to congenital heart repair to treat pulmonary ventricular pressure-volume problems and anomalous superior vena caval to left atrial connections.

Adolescent↗

An approach to direct detection of sinus nodal activity in man.

We are reporting a series of 13 out of a total of 16 patients in whom sinus nodal activity was recorded with the use of intra-atrial electrodes, during diagnostic His-bundle electrocardiography investigations. The protocol consisted of the simultaneous monitoring of the following intra-atrial points: high right atrium, low lateral right atrium, left atrium and the His-bundle electrogram (HBE), which accompanied the detecting sonde placed as a loop in the superior vena cava, with the electrodes facing the sinus nodal area. This permitted an adequate assessment of the sequence of the depolarizing activity. All patients showed sinus nodal potentials preceding the initiation of the P-wave in the surface-EKG or the atrial potential in the high right atrial lead, respectively, in the range of 60 to 270 msec depending upon the functional status of the sinus node in each patient. Such waves were clearly separated from the initiation of the atrial potential by an isoelectric line and showed functional traits compatible with the S-A nodal functions, as many authors have previously reported in experimental animal models. The text contains a review of the relevant literature and discussion upon the diagnostic significance of this method in the assessment of sinus nodal disease.

Adult↗

Influence of age on pulmonary haemodynamics at rest and during supine exercise.

To determine the effects of age on the pulmonary circulation at rest and on exercise we analysed the results of right heart catheterization studies performed in 125 asymptomatic subjects aged 14-68 years, who were healthy or had indispositions which did not impair cardiac or pulmonary function. Age accounted for less than 10% of total variation in resting values of right atrial, pulmonary artery and wedge pressures, and of cardiac output. The pulmonary artery-wedge pressure gradient and flow resistance at rest significantly increased with age. On exercise there were significant increases with age in right atrial, pulmonary artery and wedge pressures, pulmonary to wedge pressure gradient and flow resistance, but cardiac output was not influenced by age. Pulmonary circulation variables at rest are mainly influenced by sex and size, but during exercise significant effects of age are apparent.

Adolescent↗

The right heart in congenital heart disease.

The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right shunts (e.g., atrial septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot). RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary to congenital and postoperative left to right shunts or from defects that cause pulmonary venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis). The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis, or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale. Congenital heart disease, therefore, must be included in the differential diagnosis of patients who present with right ventricular dysfunction.

Journal Article↗

Characterization of myocardial protein composition in dilated cardiomyopathy by two-dimensional gel electrophoresis.

In order to identify alterations in the myocardial protein pattern that characterize dilated cardiomyopathy (DCM), we compared, by two-dimensional gel electrophoresis, right atrial protein patterns from five patients with DCM and four with normal left ventricular function (two gels per patient). Using computer assisted analysis (PDQUEST, 4.1) we found reproducible protein patterns in the 18 gels (23 x 30 cm, pH 4-9, molecular weight 10-150 kDa). In the two gels from the same patient, 91% of proteins were identical in their position in the pattern and the relative intensities of these protein species correlated with r = 0.85. Three hundred and two +/- 50 protein species were found in several gels, 186 in all 18 gels. Seven proteins in the DCM group were decreased in their relative intensity by > 100%, six were increased by > 100%. Significant quantitative differences between DCM and control patients were found for 25 protein species. Based on seven external marker proteins, a pH and molecular weight value could be calculated for each protein. So far, 30 protein species have been identified by antibodies, amino acid analysis or sequencing procedures. From the 25 proteins that are significantly different between DCM and controls, three have been identified. Expression of the mitochondrial creatine kinase and alpha cristallin B chain was significantly increased in DCM; the malate dehydrogenase family was also significantly decreased in DCM. Two-dimensional electrophoresis appears to be a powerful method for the detection of disease-associated alterations in the myocardial protein pattern.

Adult↗

Right to left interatrial communications after the modified Fontan procedure: identification and management with transcatheter occlusion.

OBJECTIVE: To describe unusual venous communications from the right to the left atrium resulting in cyanosis after the modified Fontan procedure, and their management with transcatheter occlusion. METHODS: Between September 1992 and November 1994, eight patients were assessed for persistent cyanosis after a modified Fontan procedure. Desaturation was found to be caused by unusual venous shunts originating at atrial level, and transcatheter occlusion with either a double umbrella or coil was attempted. RESULTS: Three types of venous channels were identified. The first type of communication consisted of thin long tortuous channels originating from the right atrial wall, and draining into the left atrium through a capillary network. The second type of communication was in the superior anterior portion of the atrial baffle, incorporating the pectinate muscles of the right atrium, draining into the neoleft atrium. These channels were shorter and often fanned out into small vessels toward the right atrial appendage. In each instance, the shunts were in the superior suture line of a lateral tunnel modification of the Fontan procedure. The third type of communication originated from the inferior vena cava, connecting inferior phrenic veins to pericardial veins and subsequently to the left atrium, at or close to the ostium of the left pulmonary veins. Before device occlusion, the room air aortic oxygen saturation was 88(SD 4)% (range 84% to 94%), and increased to 95(3)% (range 91% to 100%) following occlusion (PL << 0.001). The mean right atrial pressure was 14(4)mm Hg and remained unchanged after occlusion. In six patients there was complete shunt obliteration, while in two both occluded with umbrella devices, a small residual leak persisted. No complication occurred during or immediately after catheterisation. CONCLUSIONS: Unusual venous communications can evolve after the Fontan procedure, resulting in the development or persistence of cyanosis. Some of these communications may be present preoperatively as normal veins draining into the right atrium, enlarging with the increased atrial pressure after surgery. These observations affect long term function after the Fontan procedure. Transcatheter occlusion of these communications is technically feasible and effective, although recurrence may occur.

Adolescent↗

Role of active changes in venous capacity by the carotid baroreflex: analysis with a mathematical model.

To elucidate the role of venous capacity active changes in short-term cardiovascular homeostasis, a mathematical model of the carotid-sinus baroreflex system has been developed. In the model the cardiovascular system is represented as the series arrangement of six lumped compartments, which synthesize the fundamental hemodynamic properties of the systemic arterial, systemic venous, pulmonary arterial, and pulmonary venous circulations as well as of the left and right cardiac volumes. Cardiac outputs from the left and right ventricles are computed as a function of both downstream arterial pressure (afterload) and upstream atrial pressure (preload). Four distinct feedback regulatory mechanisms, working on systemic arterial resistance, heart rate, systemic venous unstressed volume, and systemic venous compliance, are assumed to operate on the cardiovascular system in response to carotid sinus pressure changes. All model parameters, both in the cardiovascular system and in feedback regulatory mechanisms, have been assigned on the basis of physiological data now available. The model is used here to simulate the pattern of the main hemodynamic quantities in the short time period (1-2 min) after acute carotid sinus activation in vagotomized subjects. Simulation results indicate that the model can reproduce experimental data quite well, with reference both to open-loop experiments and to an acute blood hemorrhage performed in closed-loop conditions. Moreover, computer simulations indicate that active changes in venous unstressed volume are of primary importance in regulating cardiac output and systemic arterial pressure during activation of the carotid sinus baroreflex.

Animals↗

Kinetics of electrophysiologic changes during oral loading of amiodarone and after withdrawal of amiodarone in the unsedated dog.

We examined the temporal kinetics of onset and offset of the cardiac electrophysiologic effects of orally administered amiodarone in chronically instrumented, unsedated adult dogs (n = 8). Right atrial (RA), atrioventricular nodal (AVN), and right ventricular (RV) effective refractory period (ERP), and AVN functional refractory period (FRP), were determined daily for 21 days during amiodarone loading (24 mg/kg/day) and for 21 days after cessation of amiodarone. Left ventricular (LV) ERP was assessed in four of eight animals. Group mean RA-ERP peaked and plateaued early during amiodarone loading (time to reach one-half observed peak change [t1/2 onset] = 1.2 +/- 0.5 days) and rapidly returned toward baseline after cessation of drug (decay time to one-half peak value [t1/2 offset] = 2.0 +/- 1.7 days). Group mean RV-ERP rose in a linear manner throughout the loading period (t1/2 onset = 9.3 +/- 2.1 days) and remained elevated after cessation of drug (t1/2 offset greater than 21.0 days). Group mean AVN-ERP and FRP exhibited temporal kinetics intermediate between those of the RA-ERP and RV-ERP, both during amiodarone loading and after cessation of the drug. Group mean LV-ERP onset kinetics (assessed in a limited number of animals, n = 4) appeared to differ from RV-ERP onset kinetics (t1/2 onset = 2.5 +/- 2.5 days), whereas LV-ERP and RV-ERP offset kinetics appeared similar (t1/2 offset greater than 21 days). In summary, our findings demonstrate that during oral loading, the temporal sequence of onset of amiodarone-induced electrophysiologic effects is site dependent. Similarly, after cessation of amiodarone, the persistence of drug-induced electrophysiologic effects is both variable and site dependent.

Administration, Oral↗

Monophasic action potential mapping in human subjects with normal electrocardiograms: direct evidence for the genesis of the T wave.

T wave concordance in the normal human electrocardiogram (ECG) generally is explained by assuming opposite directions of ventricular depolarization and repolarization; however, direct experimental evidence for this hypothesis is lacking. We used a contact electrode catheter to record monophasic action potentials (MAPs) from 54 left ventricular endocardial sites during cardiac catheterization (seven patients) and a new contact electrode probe to record MAPs from 23 epicardial sites during cardiac surgery (three patients). All patients had normal left ventricular function and ECGs with concordant T waves. MAP recordings during constant sinus rhythm or right atrial pacing were analyzed for activation time (AT) = earliest QRS deflection to MAP upstroke, action potential duration (APD) = MAP upstroke to 90% repolarization, and repolarization time (RT) = AT plus APD. AT and APD varied by 32 and 64 msec, respectively, over the left ventricular endocardium and by 55 and 73 msec, respectively, over the left ventricular epicardium. On a regional basis, the diaphragmatic and apicoseptal endocardium had the shortest AT and the longest APD, and the anteroapical and posterolateral endocardium had the longest AT and the shortest APD (p less than .05 to less than .0001). RT was less heterogeneous than APD, and no significant transventricular gradients of RT were found. In percent of the simultaneously recorded QT interval, epicardial RT ranged from 70.8 to 87.4 (mean 80.7 +/- 3.9) and endocardial RT ranged from 80 to 97.8 (mean 87.1 +/- 4.4) (p less than .001).(ABSTRACT TRUNCATED AT 250 WORDS)

Action Potentials↗

Radiofrequency catheter ablation of the atria eliminates pacing-induced sustained atrial fibrillation and reduces connexin 43 in dogs.

BACKGROUND: We assessed the effects of radiofrequency catheter ablation (RFCA) of the atrial epicardium on pacing-induced sustained atrial fibrillation (AF) and the expression and distribution of the intercellular gap junction protein connexin 43 (Cx43) in dogs. METHODS AND RESULTS: In 12 mongrel dogs, after creation of complete AV block and implantation of a ventricular inhibited pacemaker, a high-rate pulse generator (20 to 30 Hz to induce AF) was implanted in the neck, connected to a right atrial endocardial pacing lead, and used to pace the atrium for 10 to 14 weeks. In group 1 (n=9 dogs), corrected sinus node recovery time (CSNRT), P-wave duration, 24-hour Holter ECG, maximal heart rate (MHR) in response to isoproterenol, and intrinsic heart rate (IHR) after atropine (0.04 mg/kg) and propranolol were measured before and after atrial pacing and RFCA. Group 2 dogs were used to assess the effect of chronic AF alone on Cx43 expression and distribution. All group 1 dogs developed sustained (>24 hours) AF. Right-sided RFCA of the atria eliminated the sustained AF in 5 dogs, but both right and left atrial RFCA was required to abolish sustained AF in the other 4 dogs. After RFCA restored sinus rhythm, CSNRT and P-wave duration were prolonged and MHR and IHR were decreased. Chronic rapid atrial pacing (group 2) increased the expression of Cx43, which was absent in ablated areas and markedly depressed in viable atrial myocytes near the ablation zones (group 1). CONCLUSIONS: Rapid atrial pacing for long time periods induced sustained AF that can be eliminated by linear right and left atrial lesions created with RFCA, with preservation of sinus rhythm and atrial contractile function. Chronic AF increased the expression and distribution of gap junction protein Cx43, which became reduced in ablated and nearby nonablated areas.

Animals↗

Tricuspid atresia corrected with valved xenograft conduits.

In eight patients from 1976 until 1980, tricuspid atresia (TA) was corrected with valved xenograft conduits between either the right atrium and the main pulmonary artery or between the right atrium and the rudimentary right ventricle. All patients suffered from transient right-heart failure postoperatively and eventually developed normalized cardiac function throughout the first two months after operation. At recatheterization one year postoperatively, the right atrial pressure had generally only increased slightly, the pulmonary artery pressure was normal in all patients and only minor pressure differences were present across the valved conduits. X-ray examination showed normalization of the heart size in the majority of the patients, and in those with conduits between the right atrium and the right ventricle a considerable enlargement of the right ventricular chamber together with normalization of right ventricular contractility had developed. Arterial oxygen saturation, haemoglobin and haematocrit valves had normalized in all patients. One patient died postoperatively of intractable right ventricular failure, septicaemia and intravascular coagulation, and another was uneventfully re-operated on two years postoperatively because of conduit thrombosis. Clinical results were gratifying. Two patients with valved conduits between the right atrium and the right ventricle showed a normal unrestricted level of activity without medication, while patients with valved conduits between the right atrium and the main pulmonary artery were digitalized with an almost normal level of activity. Early repair with valved conduits of patients with TA is advocated.

Angiography↗

Experience with the Fontan procedure.

From 1975 to the present, 45 patients have undergone modifications of the Fontan procedure for complex congenital heart disease. There were 30 males and 15 females ranging in age from 2 to 38 years (mean 13 years). Primary diagnoses were tricuspid atresia in 19, univentricular heart in 24, and pulmonary atresia with intact ventricular septum in two. Right atrial-pulmonary arterial connections were performed in 32 patients, 11 with conduits (seven with valves and four without) and 21 by direct anastomosis with patch augmentation. Right atrial-right ventricular connections were made in 13 patients, six with valved conduits and seven without conduits. Follow-up ranged from 0.1 to 9 years, with a mean of 2.3 years. There were three early deaths (less than 30 days) (7%) and two late deaths (5%) in this series, all in patients with a univentricular heart. The late deaths were both related to venous hypertension. A venous assist device was used in eight patients in the immediate postoperative period and was effective in improving cardiac output and reducing fluid accumulation. Postoperative Doppler flow studies in 15 patients revealed biphasic pulmonary artery flow in all without distinction between the type of connection or the presence of a valve. Cardiac catheterization was performed in 16 patients a mean of 14 months postoperatively (range 1 to 42 months) and revealed a reduced cardiac index at rest. Exercise testing in eight patients demonstrated a marked rise in right atrial pressure with a reduced rise in the cardiac index, even in those without functional limitations. With a mean follow-up of 2.3 years, 78% of patients were in New York Heart Association Class I, 17% in Class II, and 5% in Class III. We conclude that the Fontan procedure is an excellent operation in carefully selected patients with tricuspid atresia and other forms of complex congenital heart disease.

Adolescent↗

Anesthetic and hemodynamic effects of the stereoisomers of medetomidine, an alpha 2-adrenergic agonist, in halothane-anesthetized dogs.

The anesthetic-sparing and hemodynamic effects of the stereoisomers of the highly selective alpha 2-adrenergic agonist medetomidine were studied in halothane-anesthetized dogs. Male beagles were anesthetized with halothane in oxygen. After a 2-hour equilibration period, halothane MAC and baseline hemodynamic functions were determined. DL-(n = 7), D- (n = 5), or L-medetomidine (n = 5) at 1, 3, and 10 micrograms/kg was administered via a right atrial port over 15 minutes while each dog was given halothane at the MAC dose for that animal. Twenty minutes after the end of infusion (when the hemodynamic variables were stable), hemodynamic function was reassessed. Halothane MAC was then redetermined. MAC for halothane significantly decreased after DL-medetomidine administration in a dose-dependent fashion to the extent that at the highest dose (10 micrograms/kg) the halothane MAC was less than 0.1%. This effect could be mimicked by the D-isomer, whereas the L-isomer was without effect. Neither isomer changed the mean arterial pressure, whereas only the D-isomer significantly decreased heart rate and cardiac output. Medetomidine, the highly selective alpha 2-adrenergic agonist, reduces the MAC for volatile anesthesia by a greater degree than with any other physiologic, pharmacologic, or pathologic intervention thus far reported. The fact that this effect is stereospecific suggests a structure activity relation that can be accounted for by a homogeneous receptor population. The role of medetomidine as a supplemental anesthetic agent appears promising and requires further investigation.

Adrenergic alpha-Agonists↗

Current status of the Maze procedure for the treatment of atrial fibrillation.

Since the first patient underwent the Maze procedure on September 25, 1987, 346 patients have undergone this operation for the treatment of atrial fibrillation. The procedure was designed as an open-heart operation performed through a median sternotomy. It underwent 2 major modifications relatively early in the series, evolving into the so-called Maze-III procedure, which has been used exclusively since April 16, 1992. Since that time, the Maze-III procedure has been adapted to allow it to be done by minimally invasive techniques. In addition, we recently performed the entire procedure in 2 patients without the use of cardiopulmonary bypass. The operative mortality rate has remained at 2% to 3%. This includes patients undergoing concomitant high-risk cardiac surgical procedures and all re-do cases. The overall success rate in curing atrial fibrillation has been 99%. The procedure itself has been shown to cause no permanent damage to the sinus node. The left atrium has been documented to function long-term postoperatively in 93% of patients and the right atrium functions in 99% of patients. The Maze-III procedure remains the surgical procedure of choice for the treatment of medically refractory atrial fibrillation.

Atrial Fibrillation↗