Seamus Cullen

Percutaneous pulmonary valve implantation impact of evolving technology and learning curve on clinical outcome

Background— Percutaneous pulmonary valve implantation was introduced in the year 2000 as a nonsurgical treatment for patients with right ventricular outflow tract dysfunction. Methods and Results— Between September 2000 and February 2007, 155 patients with stenosis and/or regurgitation underwent percutaneous pulmonary valve implantation. This led to significant reduction in right ventricular systolic pressure (from 63±18 to 45±13 mm Hg, P<0.001) and right ventricular outflow tract gradient (from 37±20 to 17±10 mm Hg, P<0.001). Follow-up ranged from 0 to 83.7 months (median 28.4 months). Freedom from reoperation was 93% (±2%), 86% (±3%), 84% (±4%), and 70% (±13%) at 10, 30, 50, and 70 months, respectively. Freedom from transcatheter reintervention was 95% (±2%), 87% (±3%), 73% (±6%), and 73% (±6%) at 10, 30, 50, and 70 months, respectively. Survival at 83 months was 96.9%. On time-dependent analysis, the first series of 50 patients (log-rank test P<0.001) and patients with a residual gradient >25 mm Hg (log-rank test P=0.01) were associated with a higher risk of reoperations. Conclusions— Percutaneous pulmonary valve implantation resulted in the ability to avoid surgical right ventricular outflow tract revision in the majority of cases. This procedure might reduce the number of operations needed over the total lifetime of patients with right ventricle–to–pulmonary artery conduits.

Ebstein's anomaly: Presentation and outcome from fetus to adult

OBJECTIVES This study was conducted to investigate the presentation and outcome of patients with Ebsteins anomaly of the tricuspid valve. BACKGROUND Ebstins anomaly may present at any age and has a highly variable clinical course. Previous natural history studies have been based on clinical and angiographic diagnosis and have included mainly older children and adults. Echocardiography, however, has facilitated fetal and neonatal diagnosis so that the natural history needs to be redefined. METHODS We reviewed 220 cases of Ebsteins anomaly presenting from fetal to adult life between 1958 and 1991, with 1 to 34 years of follow-up. RESULTS The most common presentation in each age group was abnormal routine prenatal scan for fetuses (86%), cyanosis for neonates (74%), heart failure for infants (43%), incidental murmur for children (63%) and arrhythmia for adolescents and adults (42%). Early presentation was frequently associated with other cardiac lesions, usually pulmonary stenosis or atresia. Surgery was undertaken at some stage in 86 (39%) of the 220 patients. Actuarial survival for all liveborn patients was 67% at 1 year and 59% at 10 years. There were 58 deaths, including 26 from heart failure, 19 perioperative and 8 sudden. Predictors of death included echocardiographic grade of severity at presentation (relative risk 2.7 for each increase in grade, 95% confidence limits 1.6 to 4.6), fetal presentation (6.9, confidence limits 1.6 to 16.5) and right ventricular outflow tract obstruction (2.1, confidence limits 1.1 to 4.4). Morbidity was mainly related to arrhythmias and late hemodynamic deterioration. Of 155 survivors, 129 (83%) were in functional class 1 and 104 (67%) were receiving no medical therapy. CONCLUSIONS In Ebsteins anomaly, fetal and neonatal presentation is associated with a poor outcome and can be predicted by the echocardiographic appearance and presence of associated lesions. In older children and adults, incidental findings and arrhythmia are common and the long-term outcome is superior.

Impairment of endothelium-dependent pulmonary artery relaxation in children with congenital heart disease and abnormal pulmonary hemodynamics.

BackgroundEndothelial injury may be an important event in the pathophysiology of pulmonary hypertension. We therefore investigated whether endothelial dysfunction occurs early in children with congenital heart defects who are at risk of developing pulmonary vascular disease. Methods and ResultsIn 25 children aged 3-16 years, we studied the response of the pulmonary circulation to graded infusions of acetylcholine (an endothelium-dependent vasodilator) and nitroprusside (a dilator not dependent on endothelial function). Diameter of a bronchopulmonary segment artery and pulmonary blood flow velocity were measured using quantitative angiography and intra-arterial Doppler catheters in 10 children aged 4-16 years with normal pulmonary hemodynamics (controls), seven children aged 3-12 years with left-to-right shunt lesions resulting in increased pulmonary flow, and eight children aged 3-14 years with established pulmonary vascular disease. In the controls, there was a dose-dependent increase in flow velocity in response to acetylcholine (maximal increase, 93±7%) and in response to nitroprusside (51±8%). In contrast, in patients with pulmonary vascular disease, the response of flow velocity to similar doses of acetylcholine (33±7%, p<0.01) and nitroprusside (7±13%, p<0.01) were impaired. In the patients with high pulmonary flow, there was an impaired response to acetylcholine (46+9%, v p<0.Ol), but response to nitroprusside was preserved (42±8%, p>0.10), consistent with endothelial dysfunction. Arterial diameter was unchanged during acetylcholine infusion in all subjects and increased only modestly in response to nitroprusside (10%), indicating that the major site of action of each agent is distal to the segmental pulmonary arteries. ConclusionEndothelium-dependent pulmonary artery relaxation can be demonstrated in vivo and is impaired in young patients with increased pulmonary flow secondary to congenital heart disease. This impairment may be an important early event in the pathogenesis of pulmonary vascular disease.

Outcome in neonates with Ebstein's anomaly

The presentation and outcome of 50 patients with neonatal Ebsteins anomaly seen from 1961 to 1990 were reviewed. The majority (88%) presented in the 1st 3 days of life; cyanosis (80%) was the most common presenting feature. Associated defects, present in 27 infants (54%), included pulmonary stenosis in 11 and atresia in 7. Nine patients (18%) died in the neonatal period; there were 15 late deaths (due to hemodynamic deterioration in 9, sudden death in 5 and a noncardiac cause in 1) at a mean age of 4.5 years (range 4 months to 19 years). Actuarial survival at 10 years was 61%. A new echocardiographic grade (1 to 4 in order of increasing severity of the defect) was devised with use of the ratio of the area of the right atrium and atrialized right ventricle to the area of the functional right ventricle and left heart chambers. Cardiac death occurred in 0 of 4 infants with grade 1, 1 (10%) of 10 with grade 2, 4 (44%) of 9 with grade 3 and 5 (100%) of 5 with grade 4. In a multivariate analysis of clinical and investigational features at presentation, echocardiographic grade of severity was the best independent predictor of death. Neonates with Ebsteins anomaly have a high early mortality rate and those surviving the 1st month of life remain at high risk of late hemodynamic deterioration or sudden death. Echocardiographic grading of severity of the defect permits prognostic stratification.

Prognostic significance of ventricular arrhythmia after repair of tetralogy of fallot: A 12-year prospective study

OBJECTIVE The aim of this study was to examine the prognostic significance of ventricular arrhythmia on the ambulatory electrocardiogram (ECG) after repair of tetralogy of Fallot. BACKGROUND Ventricular arrhythmia is common after repair of tetralogy of Fallot and has been proposed as the basis for late sudden death. The prognostic significance of ventricular arrhythmia on ambulatory ECG and the indications for therapy are uncertain. METHODS We performed a 48-h ambulatory ECG in 86 patients (3 to 45 years old [mean age 14 years]) after repair of tetralogy of Fallot. These patients were then followed up prospectively for 12 years. RESULTS At initial assessment in 1980, 47 patients (55%) had infrequent uniform ventricular extrasystoles (16 patients) or normal cardiac rhythm (31 patients) Group 1), and 39 patients (45%) had frequent uniform ventricular extrasystoles (> 30/h, 2 patients), complex extrasystole (30 patients) or nonsustained ventricular tachycardia (7 patients) (Group 2). There were no significant clinical or hemodynamic differences between the groups. In addition, nine patients had supraventricular tachyarrhythmia. Antiarrhythmic therapy was prescribed only for the 10 patients who had symptoms attributable to arrhythmia. There were two sudden deaths in Group 1 (4%) and one nonsudden death in Group 2 (2.5%). The absolute difference in mortality between groups was therefore 1.5% (95% confidence limits -6% to +9%), excluding a clinically significant difference in outcome. All but 1 of the 39 patients with complex ventricular arrhythmia are alive and well, including those with elevated (> or = 60 mm Hg) right ventricular pressure. CONCLUSIONS Nonsustained ventricular arrhythmia on ambulatory ECG does not identify patients at high risk for sudden death after repair of tetralogy of Fallot. There does not appear to be any advantage in potentially dangerous long-term antiarrhythmic therapy for asymptomatic postoperative patients.

Early Versus Late Functional Outcome After Successful Percutaneous Pulmonary Valve Implantation Are the Acute Effects of Altered Right Ventricular Loading All We Can Expect

OBJECTIVES The purpose of this study was to assess the potential of late positive functional remodeling after percutaneous pulmonary valve implantation (PPVI) in right ventricular outflow tract dysfunction. BACKGROUND PPVI has been shown to impact acutely on biventricular function and exercise performance, but the potential for further late functional remodeling remains unknown. METHODS Sixty-five patients with sustained hemodynamic effects of PPVI at 1 year were included. Patients were divided into 2 subgroups based on pre-procedural predominant pulmonary stenosis (PS) (n = 35) or predominant pulmonary regurgitation (PR) (n = 30). Data from magnetic resonance imaging and cardiopulmonary exercise testing were compared at 3 time points: before PPVI, within 1 month (early) and at 12 months (late) after PPVI. RESULTS There was a significant decrease in right ventricle end-diastolic volume early after PPVI in both subgroups of patients. Right ventricle ejection fraction improved early only in the PS group (51 ± 11% vs. 58 ± 11% and 51 ± 12% vs. 50 ± 11%, p < 0.001 for PS, p = 0.13 for PR). Late after intervention, there were no further changes in magnetic resonance parameters in either group (right ventricle ejection fraction, 58 ± 11% in the PS group and 52 ± 11% in the PR group, p = 1.00 and p = 0.13, respectively). In the PS group at cardiopulmonary exercise testing, there was a significant improvement in peak oxygen uptake early (24 ± 8 ml/kg/min vs. 27 ± 9 ml/kg/min, p = 0.008), with no further significant change late (27 ± 9 ml/kg/min, p = 1.00). In the PR group, no significant changes in peak oxygen uptake from early to late could be demonstrated (25 ± 8 ml/kg/min vs. 25 ± 8 ml/kg/min vs. 26 ± 9 ml/kg/min, p = 0.48). CONCLUSIONS In patients with a sustained hemodynamic result 1 year after PPVI, a prolonged phase of maintained cardiac function is observed. However, there is no evidence for further positive functional remodeling beyond the acute effects of PPVI.

Does restrictive right ventricular physiology in the early postoperative period predict subsequent right ventricular restriction after repair of tetralogy of Fallot

Objective To assess the relation between immediate postoperative right ventricular (RV) diastolic physiology and subsequent diastolic function in patients after repair of tetralogy of Fallot. Design Serial prospective echocardiographic study early after surgical repair of tetralogy of Fallot and at mid-term follow up. Setting Tertiary referral centre. Patients 34 patients who had repair of tetralogy of Fallot between 1992 and 1995 were studied. Main outcome measures Restrictive RV physiology defined as antegrade flow in the pulmonary artery in late diastole throughout the respiratory cycle. Results Sixteen of the 34 patients had early restrictive RV physiology. The need for transannular patch repair was an independent variable predictive of early restriction (odds ratio 4.3 (1.1–47), p < 0.05). Nine of 16 patients with early restriction also had restriction at follow up, while 15 of 16 patients without restrictive RV physiology continued without restriction. Early restriction was the only independent variable predictive of late restriction (odds ratio 6.0 (1.9–273), p = 0.01). Conclusions Early and mid-term restrictive RV physiology after repair of tetralogy of Fallot is related to the repair type. Although evidence for this physiology tends to resolve in the first few days after operation, it is highly predictive of subsequent abnormalities of RV diastolic function. Similarly, normal RV diastolic physiology without restriction in the immediate postoperative period persists in the mid-term and may be associated with the long term problems of progressive RV dilatation.

Transcatheter closure of a ruptured aneurysm of the sinus of Valsalva.

A ruptured aneurysm of the sinus of Valsalva in a man of 34 was closed by a transcatheter technique with a 12 mm modified Rashkind umbrella device.

Comparison of Noncontact and Electroanatomic Mapping to Identify Scar and Arrhythmia Late After the Fontan Procedure

Background— The right atrium late after the Fontan procedure is characterized by multiple complex arrhythmia circuits. We performed simultaneous electroanatomic and noncontact mapping to assess the accuracy of both systems to identify scar and arrhythmia. Methods and Results— Mapping was performed in 26 patients aged 26.8±8.9 years, 18.7±4.4 years after Fontan surgery. The area and site of abnormal endocardium defined by electroanatomic mapping (bipolar contact electrogram <0.5 mV) were compared with those defined by noncontact mapping during sinus rhythm and by dynamic substrate mapping. Contact and reconstructed unipolar electrograms at a known distance from the multielectrode array, recorded by the noncontact system simultaneously at 452 endocardial sites, were compared for morphological cross correlation, timing difference, and amplitude. Mapping of arrhythmias was performed with both systems when possible. The median patient abnormal endocardium as defined by electroanatomic mapping covered 38.0% (range 16.7% to 97.8%) of the right atrial surface area, as opposed to 60.9% (range 21.3% to 98.5%) defined by noncontact mapping during sinus rhythm and 11.9% (range 0.4% to 67.3%) by dynamic substrate mapping. A significant decrease in electrogram cross correlation (P=0.003), timing (P=0.012), and amplitude (P=0.003) of reconstructed electrograms, but not of contact electrograms (P=0.742), was seen at endocardial sites >40 mm from the multielectrode array. Successful arrhythmia mapping by electroanatomic versus noncontact mapping was superior in 15 patients (58%), the same in 6 (23%), and inferior in 5 (19%; P=0.044). Conclusions— Electroanatomic mapping is the superior modality for arrhythmia mapping late after the Fontan procedure. Noncontact mapping is limited by a significant reduction in reconstructed electrogram correlation, timing, and amplitude >40 mm from the multielectrode array and cannot accurately define areas of scar and low-voltage endocardium.

Congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular valve: Association with aortic arch obstruction

Aortic arch abnormalities are uncommon in patients with congenitally corrected transposition of the great arteries. Over a 20-year period, 10 patients with congenitally corrected transposition and Ebsteins anomaly of the systemic atrioventricular (AV) valve were identified. Five neonates had severe systemic AV valve regurgitation with severe coarctation of the aorta (n = 4) or aortic atresia (n = 1) and presented with heart failure. Four died in the neonatal period and one who had coarctation repair died 7 months postoperatively. The remaining five patients with congenitally corrected transposition and Ebsteins anomaly had mild left AV valve regurgitation; none of these had aortic arch obstruction. In neonates who have coexisting Ebsteins anomaly of the systemic AV valve and congenitally corrected transposition, obstruction to aortic arch flow is common. Severe systemic AV valve regurgitation with a morphologic systemic right ventricle may have contributed to low anterograde flow in the ascending aorta prenatally and thereby to the aortic arch abnormality.