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Mapping the conversion of atrial flutter to atrial fibrillation and atrial fibrillation to atrial flutter. Insights into mechanisms.

It is not generally believed that there is a relation between atrial flutter, thought to be due to a single reentrant circuit, and atrial fibrillation, thought to be due to simultaneously circulating multiple-reentrant wave fronts. However, there are many reasons to suggest that these rhythms are more closely related than previously thought. To test the hypothesis that the length of an area of functional block in the right atrial free wall is critical to the conversion of atrial flutter to atrial fibrillation and of atrial fibrillation to atrial flutter, we studied spontaneous and ATP-induced conversion of stable atrial flutter to sustained atrial fibrillation and spontaneous conversion of sustained atrial fibrillation to stable atrial flutter. We studied 13 episodes of the conversion of stable atrial flutter to sustained atrial fibrillation and sustained atrial fibrillation to stable atrial flutter in seven dogs with sterile pericarditis. Six episodes were spontaneous and seven were ATP related. All episodes were studied by using a multisite mapping system to record 190 unipolar electrograms (converted in the software to 95 bipolar electrograms) from the right atrial free wall along with ECG lead II. Atrial flutter induction was attempted by atrial stimulation (S1S2 or S1S2S3) or by rapid atrial pacing for > or = 20 beats from selected sites at selected rates. For both the spontaneous and the ATP-related episodes, stable atrial flutter was defined as any episode of > or = 5 minutes, and sustained atrial fibrillation was any episode of > or = 1 minute. During all the episodes of stable atrial flutter, a line of functional block with a mean length of 24 +/- 4 mm was localized on the right atrial free wall. When the previously stable line of functional block decreased to a mean of 16 +/- 3 mm (P < .05), either spontaneously or after ATP administration (40 mg i.v.), the new line of functional block was not long enough to maintain stable atrial flutter, and conversion to atrial fibrillation resulted. This shortened line of functional block continued to change and migrate over the right atrial free wall throughout sustained atrial fibrillation. These observations were similar for both spontaneous and ATP-induced conversions. When sustained atrial fibrillation evolved to stable atrial flutter, there was reformation of a long line of functional block, long enough (> or = prior length) to create a stable reentrant circuit, which then captured the right atrial free wall and subsequently both atria.(ABSTRACT TRUNCATED AT 400 WORDS)

Adenosine Triphosphate↗

Interactions within the intrinsic cardiac nervous system contribute to chronotropic regulation.

The objective of this study was to determine how neurons within the right atrial ganglionated plexus (RAGP) and posterior atrial ganglionated plexus (PAGP) interact to modulate right atrial chronotropic, dromotropic, and inotropic function, particularly with respect to their extracardiac vagal and sympathetic efferent neuronal inputs. Surgical ablation of the PAGP (PAGPx) attenuated vagally mediated bradycardia by 26%; it reduced heart rate slowing evoked by vagal stimulation superimposed on sympathetically mediated tachycardia by 36%. RAGP ablation (RAGPx) eliminated vagally mediated bradycardia, while retaining the vagally induced suppression of sympathetic-mediated tachycardia (-83%). After combined RAGPx and PAGPx, vagal stimulation still reduced sympathetic-mediated tachycardia (-47%). After RAGPx alone and after PAGPx alone, stimulation of the vagi still produced negative dromotropic effects, although these changes were attenuated compared with the intact state. Negative dromotropic responses to vagal stimulation were further attenuated after combined ablation, but parasympathetic inhibition of atrioventricular nodal conduction was still demonstrable in most animals. Finally, neither RAGPx nor PAGPx altered autonomic regulation of right atrial inotropic function. These data indicate that multiple aggregates of neurons within the intrinsic cardiac nervous system are involved in sinoatrial nodal regulation. Whereas parasympathetic efferent neurons regulating the right atrium, including the sinoatrial node, are primarily located within the RAGP, prejunctional parasympathetic-sympathetic interactions regulating right atrial function also involve neurons within the PAGP.

Animals↗

Analysis of atrial and ventricular performance by tissue Doppler imaging in patients with atrial septal defects before and after surgical and catheter closure.

OBJECTIVE: To compare the effects of surgical and device closure of atrial septal defects on atrial and ventricular performance assessed by the novel tissue Doppler derived strain rate. BACKGROUND: Despite the increasing number of transcatheter closures, there is no information comparing the effect of the transcatheter closure technique on atrial performance with that of conventional surgery. Tissue Doppler derived strain rate can effectively quantify local myocardial function independent of the overall heart motion. DESIGN AND PATIENTS: Twenty-four patients [aged 21.5 (6-70) years] with isolated atrial septal defect of the secondum type before and 1 week after surgical (n = 12) or Amplatzer Septal Occluder closure (n = 12) and 30 healthy controls [aged 26.0 (2-58) years] were studied. Atrial and ventricular strain rate curves were assessed in the middle of their corresponding lateral walls in an apical four-chamber view. The systolic, early diastolic, and late diastolic strain rates peaks were measured. RESULTS: Compared to preclosure condition, the right atrial late diastolic (P < 0.01), right ventricular systolic (P < 0.01), right ventricular early diastolic (P < 0.01), and left atrial late diastolic peak (P < 0.01) strain rates were reduced after surgery but not after Amplatzer Septal Occluder closure. The LV parameters did not significantly differ before and after atrial septal defect closure by either technique. CONCLUSIONS: In contrast to surgery, transcatheter closure of atrial septal defect preserves atrial and right ventricular function. Tissue Doppler derived strain rate can be applied to provide quantitative analysis of regional atrial and ventricular performance.

Adolescent↗

[Right heart function based on 2-dimensional echocardiographic data in heart failure patients].

The right atrium and right ventricle were examined by contrast echocardiography. The function of the right chambers was evaluated by determining the changes in the heart cavity section areas while using echocardiography during different phases of the cardiac cycle. The patients with primary pulmonary hypertension and mitral valve disease and healthy subjects were examined. It was typical of the patients with mitral valve disease to have a marked decrease in the functional parameters of the right atrial fibrillation and diminished right ventricular function indices. The indices of the functional state of the right ventricle in the patients with primary pulmonary hypertension changed to a less extent despite the fact that they had a more pronounced dilatation of the right ventricle than those with mitral valve disease.

Adult↗

Transcatheter closure of atrial septal defects improves right ventricular volume, mass, function, pulmonary pressure, and functional class: a magnetic resonance imaging study.

OBJECTIVE: To characterise prospectively by magnetic resonance imaging (MRI) changes in right ventricular (RV) volume, function, and mass after transcatheter closure of atrial septal defects (ASDs) and to evaluate the course of pulmonary pressure and functional class criteria. METHODS: In 20 patients with secundum-type ASD and dilated RV diameter, MRI was performed to quantify RV end diastolic (RVEDV) and end systolic volumes (RVESV), RV mass, tricuspid annular diameter, and RV ejection fraction before and 6 and 12 months after transcatheter closure of the ASD. RV systolic pressure was measured during follow up by transthoracic echocardiography. RESULTS: Functional class improved in the majority of patients after ASD closure. RVESV (from 81 (18) ml/m2 to 53 (15) ml/m2, p < 0.001), RVEDV (from 127 (17) ml/m2 to 99 (18) ml/m2, p < 0.001), and RV mass (from 79 (10) g to 63 (8) g, p < 0.01) decreased significantly during follow up, although tricuspid annular diameter did not. RV ejection fraction improved (by 9% compared with baseline, p < 0.05) and RV systolic pressure decreased significantly (from 33 (8) mm Hg to 24 (6) mm Hg, p < 0.001) after closure. CONCLUSION: MRI studies showed significant improvement of RV volumes, mass, and function after transcatheter closure of ASDs. Restoration of the RV leads to decreased pulmonary pressure resulting in a better functional class in the majority of patients.

Adult↗

Experimental Right Atrial Ischemia and Its Response to Stimulation with Dobutamine.

In order to analyze the repercussion of experimental isolated selected right atrial ischemia on the hemodynamics of both ventricles, we investigated the response of atrial myocardium with administration of dobutamine and evaluated the utility of transesophageal echocardiography (TEE) with Doppler in the examination of alterations produced by atrial ischemia. Ten dogs were studied with normal, diminished, and increased cardiac output with ligation of the visible atrial branches of the right coronary artery. Right atrial wall movement and peak A wave velocity of tricuspid flow registered by TEE decreased (P < 0.05). The amplitude of the right atrial A wave decreased in hemodynamic recordings (P < 0.05). No significant modifications occurred in right ventricular wall movement nor in pressures registered in right or left ventricles or cardiac output. Seventy-five minutes after atrial ischemia was induced dobutamine was administered. In dogs with incomplete ligations of atrial circulation, right atrial wall movement improved (P < 0.001), and the amplitude of the peak A wave velocity of tricuspid flow increased. In dogs with complete coronary ligation, administration of the medication produced no improvement of these variables. The findings indicate that it is possible to produce selective right atrial ischemia manifested by diminished wall movement, a diminished atrial component of tricuspid flow in TEE, and decreased amplitude of the A wave in atrial pressure recordings. The localized hemodynamic changes produced by right atrial ischemia are not related to variations in venous return when right ventricular function is normal. Apparently isolated right atrial damage from ischemia does not affect ventricular function if these remain healthy. The recuperation of atrial contractility can be demonstrated with dobutamine. Transesophageal echocardiography is a very useful technique for studying right atrial ischemia and infarction.

Journal Article↗

Pathologic elucidation of the echocardiographic features of Ebstein's malformation of the morphologically tricuspid valve in discordant atrioventricular connections.

We defined the morphology of the left atrioventricular valve in Ebstein's malformation associated with congenitally corrected transposition to elucidate the approach to diagnosis by echocardiography. We found 14 unequivocal cases out of a total of 3,720 specimens. We noted the atrial arrangement, displacement, and nature of the tricuspid leaflets, and axis of the plane of the tricuspid orifice. We constructed ratios of volume of the atrialized to the functional right ventricle, and of the right to left ventricle, as well as distances of the septal attachments of the atrioventricular junctions to the respective ventricular apices. Three specimens had abnormalities of cardiac position, and 8 had ventricular septal defect. The tricuspid valve plane was rotated 47 +/- 21 degrees from its usual position into the ventricle. The tricuspid valvar tissue was variably attached to the underlying myocardium, with the most severely affected lesion being the mural leaflet followed by the septal leaflet, and the anterior leaflet attachment the least affected. Abnormalities of the tendinous cords and the effective valvar orifice occurred in 3 specimens. The ratio of the atrialized to the functional right ventricular volume was 0.74 +/- 0.49; the ratio of the fetal right to left ventricular volume was 1.18:1 +/- 0.70:1. These data suggest that plastic repairs of the right ventricle would leave a small functional right ventricle, but that valve replacement could restore the volume of the ventricle. Thus, the plane of displacement of the valve in corrected transposition appears less amenable to 4-chamber echocardiography than other forms of Ebstein's malformation. Changes in the echocardiographic planes should display the morphology and also provide some hemodynamic information.

Adult↗

The cause of the hemodynamic disturbances in Ebstein's anomaly based on observations at operation.

Ten adult patients with Ebstein's anomaly had open-heart operation for interruption of a Kent bundle. The 4 patients in Group 1 had arrhythmias. One patient had a patent foramen ovale, which was closed. In 3 patients the Kent pathway was identified and interrupted, but in the other only the pathway's anterograde function was interrupted, leaving the patient subject to supraventricular tachycardia (SVT). The 3 patients in Group 2 had mild to moderate cyanosis and dyspnea during SVT as well as mild impairment during strenuous physical activity. Only interruption of their Kent bundles and closure of the patent foramen ovale were done. The 3 patients in Group 3 had dyspnea and cyanosis on exertion, and 1 had overt right heart failure. In each patient, obstruction between the atrialized right ventricle (RV) and functioning RV by the displaced tricuspid valve (TV) was relieved by valve excision and replacement. Interruption of the Kent bundle was successful in 2 of the 3 patients. Operation for Ebstein's anomaly is indicated when any of the following conditions are present: arrhythmias due to Kent bundles that are refractory to medical management; a defect in the atrial septum that must be closed because of a history of cyanosis or paradoxical emboli, or an arrhythmia that must be corrected by right atriotomy; and obstruction between the atrialized and functioning RV or a small functioning RV, both of which can be corrected by TV replacement.

Adolescent↗

Effect of thoracic blood volume changes on steady state cardiac output.

We have investigated the extent to which shifts of blood volume out of or into the thoracic region influence the steady state cardiac output. The systemic circulation of anesthetized dogs was replaced with an artificial circuit which stimulated the pertinent mechanical characteristics of an intact circulation. As in the normal animal, the steady state venous return was proportional to the pressure gradient for venous return (i.e, mean systemic minus right atrial pressure). Cardiac function was altered either by administration of epinephrine or by changes in left ventricular afterload. At a constant mean aortic pressure of 100 mm Hg, epinephrine administration increased the steady state cardiac output by 55%. Half of this increase resulted from the lowered mean right atrial pressure (caused by improved cardiac function); the remainder resulted from an increased mean systemic pressure (caused by the volume shift to the systemic circulation). Increases in afterload transferred sufficient volume to the heart-lung compartment to reduce significantly the mean systemic pressure and, hence, the steady state venous return. Our results indicate that the heart-lung compartment contains a significant volume which is under cardiac control. In addition to being able to alter the right atrial pressure, the heart can modulate the steady state cardiac output by adjusting the mean systemic pressure. To this degree the heart can adjust its own venous return.

Animals↗

Radiofrequency and cryoablation of atrial fibrillation in patients undergoing valvular operations.

BACKGROUND: Previous studies have shown that the maze operation can restore sinus rhythm and atrial transport function in patients with chronic atrial fibrillation. The purpose of this study was to test the feasibility of the application of radiofrequency and cryoablation as an alternative to the classic maze operation. METHODS: Twelve patients undergoing mitral valve procedures were included in this study. Radiofrequency and cryoablation were applied to create lesions in both atria to simulate the classic maze operation. RESULTS: There were two surgical deaths. At the mean follow-up of 10.25 months for the remaining 10 patients; 6 were in sinus rhythm, 2 in atrial rhythm, 1 in paroxysmal atrial tachycardia, and 1 in atrial fibrillation. Doppler echocardiography at 6-month follow-up showed emergence of biatrial transport function in 3 patients and right atrial contractility in 8. At 12-month follow-up of 5 patients, Doppler echocardiography showed biatrial transport function in 3 and right atrial contractility in 4. CONCLUSIONS: Our modified maze procedure during valvular operation is effective for achieving an acceptable success rate to restore sinus rhythm and atrial transport function in patients with chronic atrial fibrillation.

Adult↗

Abnormal right ventricular size and ventricular septal motion after atrial septal defect closure: etiology and functional significance.

Postoperative echocardiogram often demonstrate persistent right ventricular dilatation and paradoxic ventricular septal motion after repair of an atrial septal defect. To determine the prevalence, causes and significance of these echocardiographic abnormalities, 31 patients were studied with catheterization and echocardiography before and after repair of an atrial septal defect. Before operation, every patient manifested right ventricular dilatation, and all but one had abnormal septal motion. After operation, right ventricular dilatation was noted in 24 (77%) and abnormal septal motion in 21 (68%) patients despite the absence of residual left to right shunting in 30 (97%). These echocardiographic abnormalities could be correlated with age at operation and length of postoperative follow-up study but did not correlate with the degree of preoperative right ventricular enlargement or with shunt size or right ventricular pressure before or after operation. There was no associated functional deficit as demonstrated by the normal maximal oxygen consumption in all 13 patients who underwent treadmill exercise testing 5 to 38 months after operation; these patients included 9 with persistent right ventricular enlargement and abnormal septal motion.

Adolescent↗

Functional bundle branch block: discordant response of right and left bundle branches to changes in heart rate.

Faster heart rates shorten refractoriness more in some tissues than in others. This study investigates whether faster heart rates shorten relative refractoriness more in the right than left bundle branch in humans. Premature atrial stimulation at 2 or more basic cycle lengths was performed in 314 patients with no evidence of atrioventricular conduction system disease. In 10 patients, both functional right and left bundle branch block (BBB) developed with premature atrial stimulation. Functional right BBB occurred at the longer basic cycle length, and functional left BBB at the shorter cycle length in 8 patients. In 2 patients functional right and functional left BBB were present at the same cycle length, but functional left BBB occurred at a shorter premature atrial coupling interval. For all patients, the mean functional right bundle branch relative refractoriness was 438 ms at a basic cycle length of 847 ms, and functional left bundle branch relative refractoriness was 357 ms at a cycle length of 622 ms (p less than 0.01). The HV interval was 45 +/- 15 ms at control and increased with functional left BBB to 77 +/- 19 ms (p less than 0.01), but not with functional right BBB. Thus, relative refractoriness of the right and left bundle branches are rate-dependent and discordant. At longer cycle lengths, relative refractoriness of the right bundle branch is greater than that of the left bundle branch, and at shorter cycle lengths relative refractoriness of the left bundle branch is greater than that of the right bundle branch. The relative refractory period curves "cross over" and can explain the presence of both functional right and left BBB in the same patient.

Adolescent↗

Negative influences of ascites on the cardiac function of cirrhotic patients.

Right and left ventricular function was evaluated in 21 men with cirrhosis and tense ascites during staged removal of ascitic fluid. During paracentesis it was observed (1) that there was a significant increase in cardiac output, stroke volume, right and left ventricular stroke work and mean rate of systolic ejection; (2) that up to a certain stage of drainage (about 5,000 ml), there was a relationship between the amount of fluid removed and the intraabdominal and right atrial pressures and (3) that there was a direct relationship between improvement of cardiac function and normalization of right atrial pressure. It is believed that the increased intra-abdominal hydrostatic pressure acting upon the diaphragm affects the intrathoracic pressure to such an extent that the transmural filling pressure of the heart is reduced, and the mean pressure and respiratory pulsations of the right atrium increased, all of which impede venous return. Improved cardiac function during paracentesis appears to be due to an augmented filling of the heart and to a larger venous return.

Adult↗

Role of anatomic architecture in sustained atrial reentry and double potentials.

To determine the role of anatomic architecture in atrial flutter, electrophysiologic findings were correlated with anatomic features in a modified model of atrial flutter with ligation of the crista terminalis. Crista ligation in the middle right atrium prolonged intraatrial conduction time in a rate-dependent manner in 12 dogs, particularly in the low right atrium. With burst atrial pacing, unidirectional block occurred either in the low right atrium or in the interatrial septal region near the superior vena cava, leading to initiation of atrial flutter. Atrial activation mapping revealed a slow conduction area in the low right atrium where conduction had been delayed by crista ligation. On the intact tissues between the venae cavae, double potentials were recorded, a finding indicative of functional block in the center of the reentrant circuit. The interdeflection time of double potentials changed with the activation sequence of atrial flutter. This change could be explained by assuming that the functional center of the reentrant circuit leaned on the right atrial free wall side. Anatomic study demonstrated that areas of slow conduction, unidirectional block, and functional block in the center of the reentrant circuit were closely related to the location of the intact crista terminalis. In conclusion, the intact portion of the crista terminalis played an important role in the genesis of atrial flutter after blockage of longitudinal conduction through the crista.

Animals↗

Right ventricular responses to vagus stimulation of fibers to discrete cardiac regions in dog hearts.

To investigate the role on the right ventricular function of the parasympathetic nerves in discrete cardiac regions, we studied the effects of stimulation of the intracardiac parasympathetic nerves to the atrioventricular (AV) nodal area (AVPS) or sinoatrial (SA) nodal area (SAPS), and stimulation of the cervical vagus nerves (CVS) on the right atrial and ventricular function in the autonomically decentralized heart of the anesthetized dog. AVPS prolonged AV conduction time (AVCT) and decreased the ventricular rate without changes in atrial rate, right atrial 'a' wave pressure (RAP), its first derivative (RAd P/dt), right ventricular pressure (RVP) and its first derivative (RVd P/dt) in the spontaneously beating heart. On the other hand, AVPS prolonged the AVCT in the atrium paced electrically and further decreased the ventricular rate with decreases in ventricular pressure responses but not atrial responses. SAPS decreased atrial and ventricular rates without AVCT changes and attenuated the right atrial and ventricular pressure responses. In the atrial paced heart, SAPS decreased the ventricular responses less than those responses in the spontaneously beating heart with similar decreases in the atrial pressure responses, but it changed neither the AVCT nor ventricular rate. On the other hand, CVS decreased the atrial and ventricular rate or pressure responses, and increased the AVCT. CVS in the paced heart also decreased the atrial and ventricular pressure responses with the prolongation of AVCT. The cardiac responses to parasympathetic stimulations were abolished by atropine. These results suggest that AVPS decreases ventricular rate without direct right ventricular pressure effects but the negative chronotropic effects of AVPS and SAPS decrease the ventricular pressure responses rate-dependently in the dog heart.

Animals↗

Coronary reperfusion enhances recovery of atrial natriuretic peptide secretion. Salvaging endocrine function in patients with acute right ventricular infarction.

BACKGROUND: The heart has been demonstrated not only to be a pumping organ but also an endocrine organ secreting atrial natriuretic peptide (ANP). We hypothesized that myocardial ischemia may affect ANP secretion and that reperfusion therapy for acute myocardial infarction can preserve endocrine function of the heart. METHODS AND RESULTS: Twenty patients with acute right ventricular infarction were examined who underwent reperfusion therapy on admission. These patients had proximal occlusion of the dominant right coronary artery involving the right atrial branches: 9 patients with successful reperfusion (SRP group) and the remaining 11 patients with unsuccessful reperfusion (URP group). Within 24 hours after the onset of infarction, a volume loading test was performed after reperfusion therapy with measurements for plasma ANP levels and hemodynamics. Before the volume loading test, the plasma ANP level and mean right atrial pressure were similar between these two groups. However, in the URP group, percent increase in ANP in response to volume loading was strikingly smaller (URP, 45 +/- 18% versus SRP, 133 +/- 25%; P < .01) despite similar percent increase in mean right atrial pressure (URP, 100 +/- 46% versus SRP, 86 +/- 23%). The peak ANP level occurred significantly later in the URP group (69 +/- 16 hours) than in the SRP group (28 +/- 9 hours, P < .001) after the onset of infarction. CONCLUSIONS: The response of ANP release to volume loading is attenuated in patients with right ventricular infarction without coronary reperfusion. However, successful reperfusion induces a rapid recovery of cardiac endocrine function as well as its mechanical function. A sufficiently elevated plasma ANP level may be a useful predictor of hemodynamic improvement in patients with right ventricular infarction.

Aged↗

Right atrial compression related to defibrillator patches.

Acceptable function of an internal defibrillator can be achieved with different patch orientations. For patients requiring defibrillator patches concomitant with a cardiac procedure requiring extracorporeal circulation, application of one of the patches within the pericardium adjacent to the right atrium has provided excellent defibrillation thresholds. We describe 4 such patients in whom a compressing thrombus subsequently developed between the patch and the atrium. The thrombus was small and asymptomatic in 1 patient, but caused localized tamponade requiring reexploration in 2 patients and a fatal superior vena caval obstruction in 1. The precise etiology of this serious complication remains unclear, but its occurrence argues against the application of intrapericardial defibrillator patches in this orientation.

Aged↗

Current surgical therapy for Ebstein anomaly in neonates.

OBJECTIVE: Neonates with profound heart failure resulting from Ebstein anomaly have historically had poor outcomes. We report our institutional experience with the surgical management of Ebstein anomaly in severely symptomatic neonates. METHODS: A retrospective review of all patients (n = 16) undergoing neonatal intervention for Ebstein anomaly between 1992 and 2005 has been carried out. The indications for operation were overt heart failure, cyanosis, and acidosis associated with tricuspid regurgitation, depressed right ventricular function, and severe cardiomegaly. The magnitude of cardiac enlargement was assessed by cardiothoracic ratio and Great Ormond Street ratio (area of right atrium + atrialized right ventricle/area of functional left atrium + left ventricle). The operative strategy was first to assess for the possibility of tricuspid valve repair with or without right ventricular outflow tract reconstruction. If this was not feasible, then right ventricular exclusion was performed by oversewing the tricuspid valve with a pericardial patch. A reduction atrioplasty was done and, depending on the extent of the atrialized portion of the right ventricle, plication was performed. A modified Blalock-Taussig shunt provided pulmonary blood flow. This univentricular approach (Starnes procedure) evolved to include a fenestration in the tricuspid valve patch to allow for right ventricular decompression. Analysis included overall and group-specific survival as well as the testing of perioperative clinical, morphologic, and surgical variables for correlation with mortality and morbidity. RESULTS: Mean age and weight at operation were 8 +/-10 days and 3.1 +/- 0.4 kg. Tricuspid valve repair was undertaken in 3 patients with 1 requiring conversion to right ventricular exclusion 3 months after the initial operation. In those with right ventricular exclusion, the tricuspid valve patch was fenestrated in 10 and nonfenestrated in 3. One patient had heart transplant as the initial procedure. There were 5 hospital deaths (31%) and no late deaths among the survivors. Survival in the cohort with a fenestrated tricuspid valve patch was 80% (8/10) versus 33% (1/3) for the nonfenestrated group. This difference did not reach statistical significance, although the trend seems clinically important. There was no difference in the cardiothoracic ratio (0.82 fenestrated vs 0.84 nonfenestrated: P = .802) or the Great Ormond Street ratio (1.2 fenestrated vs 1.02 nonfenestrated: P = .477) between the two groups. Among the 9 survivors of right ventricular exclusion, 3 have had completion of their Fontan, and all 9 have undergone a bidirectional Glenn procedure. All operations including homograft placement in the right ventricular outflow tract, whether during repair or during right ventricular exclusion, ended in death. CONCLUSION: Right ventricular exclusion with a fenestrated tricuspid valve patch combined with right atrioplasty and right ventriculoplasty and a Blalock-Taussig shunt (Starnes procedure) has provided effective palliation for neonates presenting with critical Ebstein anomaly and a tricuspid valve that cannot be repaired.

Cardiac Surgical Procedures↗