Left-sided Cardiac Lesions Research Articles

Postoperative Morbidity and Interstage Hemodynamics Following Stage I Palliation in Patients with Turner Syndrome and Hypoplastic Left Heart Syndrome.

Turner syndrome (TS) is associated with left-sided cardiac lesions, including hypoplastic left heart syndrome (HLHS). Mortality as high as 80-90% has been reported following stage I single-ventricle palliation (S1P) in patients with TS and HLHS (TS + HLHS). The specific factors that relate to poor outcomes are not well understood. This is a single-center, retrospective cohort study that includes 197 patients with HLHS who underwent S1P between 2008 and 2022. The clinical outcomes and interstage hemodynamics of TS + HLHS patients (N = 11) were compared with HLHS without TS (TS-HLHS), (N = 186). Of the 11 TS + HLHS patients, 10 underwent S1P; 4 underwent Glenn and 1 had hemodynamics considered prohibitive for Glenn; only 1 survived to Fontan palliation. Post-S1P mortality was higher in TS + HLHS (60 v 25%, p = 0.017). Following S1P, TS + HLHS had higher rates of postoperative ECMO (70 v 28%, p = 0.006), surgical necrotizing enterocolitis (20 v 3%, p = 0.007), peritoneal drain placement (70 v 31%, p = 0.012), urinary tract infection (30 v 9%, p = 0.035), and ICU readmissions (median 5 v 1, p = 0.035). Interstage hemodynamics demonstrated higher right ventricular end diastolic, (11 v 8mmHg, p = 0.033), mean pulmonary artery (20 v 13mmHg) (p = 0.002), and left atrial pressures (9 v 6mmHg, p = 0.047) in TS + HLHS. High mortality rates are described in TS + HLHS patients following S1P. In our cohort, despite most surviving more than 30 days post-S1P, long-term survival remained poor. Interstage catheterization data suggest poor physiologic candidacy for subsequent stages of single-ventricle palliation. Understanding the clinical and hemodynamic factors related to poor outcomes in TS + HLHS will help inform management for this population.

Ultrasound-Guided Radial Arterial Catheterization in Patients With Regurgitant and Stenotic Left-Sided Cardiac Valvular Lesions.

Background. The pulse characteristics of arterial circulation might affect radial artery catheterization's success rate. Therefore, we hypothesized that the success rate of radial artery catheterization would be lower in the left-sided severe stenotic valvular lesion group than severe regurgitant valvular lesion group. Methods. This prospective study was conducted among patients with left-sided cardiac valvular lesions undergoing cardiac and non-cardiac surgery. The patients with left-sided severe valvular stenosis and left-sided severe valvular regurgitation were included in the study. Radial artery cannulation was performed using an ultrasound-guided out-of-plane short-axis approach. The outcome measures were success rate, number of attempts, and cannulation time. Result. One hundred fifty-two patients were recruited for the study, and all were eligible for final analysis. The first attempt success rate was non-significantly higher in the stenotic valvular lesion group than the regurgitant group (69.7% vs 56.6%; P = .09). Furthermore, the number of attempts (median; 95% CI) was significantly higher in the regurgitant group (1; 1.2-1.43 vs 1; 1.38-1.67; P = .04). However, it may not be of clinical relevance. Moreover, the cannulation time and the number of redirections of the cannula were comparable. Heart rate was significantly higher in the regurgitant group (91.8 ± 13.9 vs 82.26 ± 15.92 beats/min; P = .00), while the incidence of atrial fibrillation was significantly higher in the stenotic lesion (P = .00). No failure was reported, and the incidence of periarterial hematoma was comparable. Conclusion. The success rate of ultrasound-guided radial arterial catheterization is comparable in left-sided stenotic valvular and regurgitant lesion groups.

Association between maternal oxygenation and brain growth in fetuses with left-sided cardiac obstructive lesions.

Impaired brain growth has been observed in fetuses with left-sided obstructive lesions (LSOL). Maternal oxygenation (MO) can alter fetal cerebral oxygenation and vascular reactivity. Our aim was to observe whether brain growth improves during MO in fetuses with LSOL. Forty-six fetuses with LSOL and 23 control fetuses were enrolled in this prospective longitudinal study. Fetuses with LSOL were subgrouped into those with MO (LSOL-MO, n = 23) and those without MO (LSOL-nMO, n = 23). Fetal head circumference (HC) and total intracranial volume (TIV) were evaluated serially at 4-week intervals. Brain biometry and growth were analyzed using linear mixed models adjusted for gestational age and sex. Spearman's correlation coefficients were calculated to identify baseline characteristics predictive of brain growth in the LSOL-MO group. Duration of MO therapy had significant interaction effects on cerebral biometry in fetuses with LSOL. TIV increased more rapidly after 8 weeks of oxygen exposure and HC was larger after 16 weeks of oxygen exposure in the LSOL-MO group compared with the LSOL-nMO group (P < 0.001). The change in TIV at the final time- point relative to the initial timepoint in the LSOL-MO group correlated negatively with the baseline pulsatility index of the middle cerebral artery (r = -0.58, P = 0.003) and baseline myocardial performance index of the left ventricle (r = -0.68, P < 0.001). TIV and HC increased faster in fetuses with LSOL which had MO compared with those that did not. Lower cerebral vascular resistance and preserved left heart function at baseline may predict greater cerebral biometric growth during MO. Additional research, including larger serial studies, is needed to confirm these preliminary findings and evaluate the clinical application of MO in this population. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Outcome and Impact of Associated Left-Sided Cardiac Lesions in Coarctation of the Aorta Diagnosed During Fetal Life

Significant effort has been put into the optimization of the antenatal diagnosis of coarctation of the aorta (CoA). However, although left-sided cardiac lesions are known to cluster, the necessity to intervene postnatally for other left-sided cardiac lesions has not been reported in a cohort of fetuses with suspected CoA. We report a study of all 89 fetuses with antenatally suspected and postnatally confirmed diagnosis of CoA who underwent CoA repair as the primary procedure at a single tertiary congenital heart disease center over 10 years (January 1, 2010, to December 31, 2019). Almost 1 in 5 patients (18%) had to undergo surgery and/or transcatheter intervention on additional left-sided cardiac lesions (14%) and/or reintervention on the aortic arch (12%) during follow-up to median age of 2.85 years. Freedom from intervention at 5 years was 78% (95% confidence interval [CI] 67 to 88%) if reintervention on CoA was excluded, and 72% (95% CI 60 to 82%) if this was included. Five-year survival was 95% (95% CI 90 to 100%). Furthermore, 20% of affected infants had genetic (10%) and/or extracardiac (16%) abnormalities. Our study highlights the need for comprehensive antenatal counseling, including the prognosis of primary repair of CoA and the potential development of additional left-sided cardiac lesions, which may be difficult to diagnose prenatally even in expert hands or impossible to diagnose because of the physiology of the fetal circulation.

Valvular Heart Disease in Pregnancy.

The number of reproductive age women with valvular heart disease is rising and accounts for one third of all heart disease among pregnant women. Severe, symptomatic left-sided cardiac lesions, particularly mitral and aortic stenosis, and mechanical heart valves, are associated with adverse maternal and fetal outcomes. Decreasing maternal and fetal risk requires shared decision-making among patients and the heart team, consisting of obstetricians, maternal-fetal medicine subspecialists, and cardiologists.

Novel variants in CDH2 are associated with a new syndrome including Peters anomaly.

Peters anomaly (PA) is a congenital corneal opacity associated with corneo-lenticular attachments. PA can be isolated or part of a syndrome with most cases remaining genetically unsolved. Exome sequencing of a trio with syndromic PA and 145 additional unexplained probands with developmental ocular conditions identified a de novo splicing and three novel missense heterozygous CDH2 variants affecting the extracellular cadherin domains in four individuals with PA. Syndromic anomalies were seen in three individuals and included left-sided cardiac lesions, dysmorphic facial features, and decreasing height percentiles; brain magnetic resonance imaging identified agenesis of the corpus callosum and hypoplasia of the inferior cerebellar vermis. CDH2 encodes for N-cadherin, a transmembrane protein that mediates cell-cell adhesion in multiple tissues. Immunostaining in mouse embryonic eyes confirmed N-cadherin is present in the lens stalk at the time of separation from the future cornea and in the developing lens and corneal endothelium at later stages, supporting a possible role in PA. Previous studies in animal models have noted the importance of Cdh2/cdh2 in the development of the eye, heart, brain, and skeletal structures, also consistent with the patient features presented here. Examination of CDH2 in additional patients with PA is indicated to confirm this association.

Use of percutaneous carotid artery access for performing pediatric cardiac interventions: Single-center study

Background:Carotid artery (CA) access allows a more straight route for many left heart lesions. This has previously been achieved via a surgical cut-down approach in certain pediatric cardiac interventions. However, there are little data considering CA access in pediatric cases, percutaneously.Aim:We hypothesized that there would be notable improvements in efficiency as well as overall success when using the CA for access in selected cases.Methods:Between November 2016 and January 2019, records of patients undergoing attempted percutaneous CA access under ultrasound guidance for cardiac catheterization were reviewed.Results:Thirty patients underwent 36 catheterizations; median age 17 days (range, 6 days–9 months) and median weight 3.2 kg (1.2–7.8). Procedures performed were stenting or stent redilatation of the patent ductus arteriosus in 23, stenting or angioplasty of modified Blalock–Taussig shunts in four, aortic valvuloplasty in three, angioplasty for coarctation of the aorta in four, renal angioplasty in one, and diagnostic catheterization in one case. The intended intervention was unsuccessful in two patients despite successful CA access. Follow-up imaging showed a normal carotid in 28 of 30 (94%), with mild luminal narrowing with normal Doppler velocities in two instances. No patient had clinically apparent neurological sequelae attributable to CA access.Conclusions:Our data indicate that CA access should be employed when dealing with a select group of infants requiring vertical approach for left-sided cardiac lesions. Percutaneous CA access, even in very small preterm infants, is safe and feasible with negligible vascular injury and no neurological adverse events.

Fetal Echocardiographic Parameters and Surgical Outcomes in Congenital Left-Sided Cardiac Lesions.

This study aimed to evaluate fetal echocardiographic parameters associated with neonatal intervention and single-ventricle palliation (SVP) in fetuses with suspected left-sided cardiac lesions. Initial fetal echocardiograms (1/2002-1/2017) were interpreted by the contemporary fetal cardiologist as coarctation of the aorta (COA), left heart hypoplasia (LHH), hypoplastic left heart syndrome (HLHS), mitral valve hypoplasia (MVH) ± stenosis, and aortic valve hypoplasia ± stenosis (AS). The cohort comprised 68 fetuses with suspected left-sided cardiac lesions (COA n = 15, LHH n = 9, HLHS n = 39, MVH n = 1, and AS n = 4). Smaller left ventricular (LV) length Z score, aortic valve Z score, ascending aorta Z score, and aorta/pulmonary artery ratio; left-to-right shunting at the foramen ovale; and retrograde flow in the aortic arch were associated with the need for neonatal intervention (p = 0.005-0.04). Smaller mitral valve (MV) Z score, LV length Z score, aortic valve Z score, ascending aorta Z score, aorta/pulmonary artery ratio, and LV ejection fraction, as well as higher tricuspid valve-to-MV (TV/MV) ratio, right ventricular-to-LV (RV/LV) length ratio, left-to-right shunting at the foramen ovale, abnormal pulmonary vein Doppler, absence of prograde aortic flow, and retrograde flow in the aortic arch were associated with SVP (p < 0.001-0.008). The strongest independent variable associated with SVP was RV/LV length ratio (stepwise logistical regression, p = 0.03); an RV/LV length ratio > 1.28 was associated with SVP with a sensitivity of 76% and specificity of 96% (AUC 0.90, p < 0.001). A fetal RV/LV length ratio of > 1.28 may be a useful threshold for identifying fetuses requiring SVP.

Levoatrial cardinal vein with normal left ventricle: A forgotten cause of pulmonary arterial hypertension.

Levoatrial cardinal vein (LACV) is anomalous connection between left atrium or pulmonary veins and systemic veins such as innominate vein or superior vena cava. This persistence of splanchnic circulation occurs when there is left-sided obstructive cardiac lesions such as hypoplastic left heart or mitral atresia. In this report we present three cases of LACV with well-developed left heart, without any obstructive lesions. All our cases presented with pulmonary arterial hypertension (PAH) and had associated intracardiac shunt such as ventricular/atrial septal defect and supracardiac partial anomalous pulmonary venous connection. Apart from the above shunts, LACV contributed to PAH in these cases. It is important to detect and report LACV as this may require surgical correction along with other defects. If LACV goes undetected during imaging workup, it may cause persistent PAH postoperatively.

Assessment of large copy number variants in patients with apparently isolated congenital left-sided cardiac lesions reveals clinically relevant genomic events.

Congenital left-sided cardiac lesions (LSLs) are a significant contributor to the mortality and morbidity of congenital heart disease (CHD). Structural copy number variants (CNVs) have been implicated in LSL without extra-cardiac features; however, non-penetrance and variable expressivity have created uncertainty over the use of CNV analyses in such patients. High-density SNP microarray genotyping data were used to infer large, likely-pathogenic, autosomal CNVs in a cohort of 1,139 probands with LSL and their families. CNVs were molecularly confirmed and the medical records of individual carriers reviewed. The gene content of novel CNVs was then compared with public CNV data from CHD patients. Large CNVs (>1 MB) were observed in 33 probands (∼3%). Six of these were de novo and 14 were not observed in the only available parent sample. Associated cardiac phenotypes spanned a broad spectrum without clear predilection. Candidate CNVs were largely non-recurrent, associated with heterozygous loss of copy number, and overlapped known CHD genomic regions. Novel CNV regions were enriched for cardiac development genes, including seven that have not been previously associated with human CHD. CNV analysis can be a clinically useful and molecularly informative tool in LSLs without obvious extra-cardiac defects, and may identify a clinically relevant genomic disorder in a small but important proportion of these individuals.

Heart Failure Caused by Congenital Left-Sided Lesions

There are diverse mechanisms by which congenital left-sided cardiac lesions can precipitate heart failure. Left heart outflow obstruction can impose abnormal pressure load on the left ventricle, inducing adverse remodeling, hypertrophy, and diastolic and systolic dysfunction. Abnormalities in left ventricular inflow can increase pulmonary venous pressure and predisposing to pulmonary edema. In addition, inborn abnormalities in left ventricular myocardial structure and function can impair both systolic and diastolic function and manifest as heart failure later in life. In this article, the different mechanisms, outcomes, and treatments of heart failure in patients with congenital left-sided lesions are discussed.

The Prevalence of 16p12.1 Microdeletion in Patients with Left-sided Cardiac Lesions

Left-sided cardiac lesions have a birth prevalence of approximately 1 in 1000 and have been shown to be heritable in pedigree studies. A large microdeletion at 16p12.1 is associated with childhood developmental delay, and initial studies describing this deletion identified left-sided lesions as an enriched phenotype compared with a control population. The aim of this study is to determine whether patients with left-sided cardiac lesions have an increased frequency of 16p12.1 microdeletions as compared with control populations. A cohort of 262 probands with left-sided lesions, including 53 with isolated aortic stenosis/bicuspid aortic valve, 83 with coarctation of the aorta with or without aortic stenosis/bicuspid aortic valve, and 126 with hypoplastic left heart syndrome were assessed for copy number variation at 16p12.1. The control cohort included 595 patients with conotruncal defects as a cardiac control and 971 healthy children. We detected one patient in the left-sided lesion cohort with a large duplication partially overlapping the reported 16p12.1 microdeletion, along with one patient each in the conotruncal and control cohorts with a deletion in the same region. None of these patients had dysmorphic features, extracardiac malformations, or developmental delay. In our cohort, structural variation at 16p12.1 was not identified with increased frequency in patients with left-sided lesions as compared with controls.

Evaluation of heterogeneity in the association between congenital heart defects and variants of folate metabolism genes: Conotruncal and left-sided cardiac defects

Genetic variation in the folate metabolic pathway may influence the risk of congenital heart defects. This study was undertaken to assess the associations between the inherited and maternal genotypes for variants in folate-related genes and the risk of a composite heart phenotype that included two component phenotypes: conotruncal heart defects (CTDs) and left-sided cardiac lesions (LSLs). Nine folate-related gene variants were evaluated using data from 692 case-parent triads (CTD, n = 419; LSL, n = 273). Log-linear analyses were used to test for heterogeneity of the genotype-phenotype association across the two component phenotypes (i.e., CTD and LSLs) and, when there was no evidence of heterogeneity, to assess the associations of the maternal and inherited genotypes with the composite phenotype. There was little evidence of heterogeneity of the genotype-phenotype association across the two component phenotypes or of an association between the genotypes and the composite phenotype. There was evidence of heterogeneity in the association of the maternal MTR A2756G genotype (p = 0.01) with CTDs and LSLs. However, further analyses suggested that the observed associations with the maternal MTR A2756G genotype might be confounded by parental imprinting effects. Our analyses of these data provide little evidence that the folate-related gene variants evaluated in this study influence the risk of this composite congenital heart defect phenotype. However, larger and more comprehensive studies that evaluate parent-of-origin effects, as well as additional folate-related genes, are required to more fully explore the relation between folate and congenital heart defects.

Late Outcomes of Reintervention on the Descending Aorta After Repair of Aortic Coarctation

After repair of aortic coarctation, patients may develop restenosis, aneurysms, and pseudoaneurysms at the site of prior repair. We assessed the outcomes of late reintervention on the descending aorta after aortic coarctation repair. From March 1954 to July 2008, 130 patients had operations or endovascular procedures on the descending aorta after previous coarctation repair. We excluded patients who had complex left-sided cardiac lesions or interrupted aortic arch. Mean age at reintervention was 32±24 years and 28% were female. The interval between coarctation repair and reintervention was 17±13 years. Seventy-four percent of patients had hypertension. Reasons for reintervention were restenosis (n=122 [94%]), aneurysm (n=4 [3%]), and pseudoaneurysm (n=4 [3%]). Ninety-five patients (73%) underwent operative procedures including an extra-anatomic conduit (n=41), patch repair (n=32), interposition graft (n=14), end-end anastomosis (n=6), and subclavian flap (n=2). Thirty-five patients underwent endovascular treatment (balloon dilatation, n=22 or stenting, n=13). There was no early mortality. In the surgical group, 5 patients required early reoperation for bleeding and 5 patients had early vocal cord paralysis. One patient in the endovascular group had aortic rupture at the time of intervention requiring urgent operation. Survival was 97% at 10 years. At 5 years, freedom from a second repeat procedure on the descending aorta was 96% in the surgical group and 72% in the endovascular group (P<0.001). Five years after reintervention, fewer patients required treatment for hypertension (57% versus 74%, P<0.001) and a median of 1 antihypertensive medication was prescribed compared with a median of 2 medications preintervention. Operative and endovascular management of recoarctation can be performed safely with good late outcomes.

Atrial Arrhythmias in Adult Patients With Right- Versus Left-Sided Congenital Heart Disease Anomalies

Atrial arrhythmias (AAs) are a common complication in adult patients with congenital heart disease. We sought to compare the lifetime prevalence of AAs in patients with right- versus left-sided congenital cardiac lesions and their effect on the prognosis. A congenital heart disease diagnosis was assigned using the International Disease Classification, Ninth Revision, diagnostic codes in the administrative databases of Quebec, from 1983 to 2005. Patients with AAs were those diagnosed with an International Disease Classification, Ninth Revision, code for atrial fibrillation or intra-atrial reentry tachycardia. To ensure that the diagnosis of AA was new, a washout period of 5 years after entry into the database was used, a period during which the patient could not have received an International Disease Classification, Ninth Revision, code for AA. The cumulative lifetime risk of AA was estimated using the Practical Incidence Estimators method. The hazard ratios (HRs) for mortality, morbidity, and cardiac interventions were compared between those with right- and left-sided lesions after adjustment for age, gender, disease severity, and cardiac risk factors. In a population of 71,467 patients, 7,756 adults developed AAs (isolated right-sided, 2,229; isolated left-sided, 1,725). The lifetime risk of developing AAs was significantly greater in patients with right- sided than in patients with left-sided lesions (61.0% vs 55.4%, p <0.001). The HR for mortality and the development of stroke or heart failure was similar in both groups (HR 0.96, 95% confidence interval [CI] 0.86 to 1.09; HR 0.94, 95% CI 0.80 to 1.09; and HR 1.10, 95% CI 0.98 to 1.23, respectively). However, the rates of cardiac catheterization (HR 0.63, 95% CI 0.55 to 0.72), cardiac surgery (HR 0.40, 95% CI 0.36 to 0.45), and arrhythmia surgery (HR 0.77, 95% CI 0.6 to 0.98) were significantly less for patients with right-sided lesions. In conclusion, patients with right-sided lesions had a greater lifetime burden of AAs. However, their morbidity and mortality were no less than those with left-sided lesions, although the rate of intervention was substantially different.

Functional Tricuspid Regurgitation in Mitral Valve Disease: Epidemiology and Prognostic Implications

In this review we summarize the data on epidemiology and natural history of functional tricuspid valve regurgitation as it applies to surgery for mitral valve disease. Tricuspid regurgitation in the context of mitral valve disease is frequent and is associated with substantial reduction in survival and quality of life. In many patients, the correction of left-sided cardiac lesions does not lead to resolution of tricuspid regurgitation. Significant tricuspid regurgitation after mitral valve surgery portends a poor prognosis, a course that is often not altered by subsequent surgical therapy. Although a liberal approach to tricuspid annuloplasty is widely practiced, the evidence that this approach alters the natural history of functional tricuspid regurgitation is not yet available, so it is not certain how much of the negative impact of tricuspid regurgitation is causative, rather than confounding, and to what degree we will improve long-term outcomes of mitral valve surgery by liberal tricuspid annuloplasty.

The Anesthetic Management for Cesarean Delivery in a Patient with Shone's Syndrome

Shone's syndrome is a rare congenital cardiac condition that consists of up to four obstructive left-sided cardiac lesions. We report a 17-yr-old nullipara with Shone's syndrome who presented for cesarean delivery. She had mild mitral stenosis and mild left ventricular outflow tract obstruction. Epidural anesthesia was attempted, but the patient developed severe hypotension associated with fetal bradycardia necessitating immediate cesarean delivery under general anesthesia. The peripartum anesthetic and management considerations for these patients are discussed.

Frequency of Aberrant Subclavian Artery, Arch Laterality, and Associated Intracardiac Anomalies Detected by Echocardiography

Tetralogy of Fallot is generally considered to be the most common congenital heart defect associated with an aberrant subclavian artery (ASA), but the prevalence of ASA in patients with other cardiac anomalies is not well described. The pediatric echocardiography database, with 15,871 initial echocardiograms, was searched for all patients with ASA. Arch laterality and associated intracardiac anomalies were documented for each patient. ASA was found in 226 patients, of whom 171 had a left aortic arch (LAA) and 55 had a right aortic arch (RAA). The occurrence of ASA was 1% in patients with LAA (171 of 15,650) and 25% in patients with RAA (55 of 221; p = 0.001). Intracardiac anatomy was normal in 32% of patients with ASA/LAA and 25% with ASA/RAA. Conotruncal anomalies occurred more frequently with ASA/RAA than ASA/LAA (36% vs 18%; p = 0.01). Atrioventricular canal defects accounted for 10% and left-sided cardiac obstructive lesions accounted for 11% of subjects with ASA/LAA. ASA was rarely associated with d-transposition of the great arteries (1 of 226) and double-outlet right ventricle (5 of 226). The prevalence of ASA was highest in patients with interrupted aortic arch (11 of 38; 29%). In patients with tetralogy of Fallot, the overall prevalence of ASA was 8% (34 of 447), but was higher with RAA (16 of 103; 16%). The highest prevalence of ASA occurred in the subgroup of patients with tetralogy of Fallot with pulmonary atresia and RAA (6 of 25; 24%). In conclusion, ASA was more common in patients with RAA, especially with conotruncal anomalies. In patients with LAA, hypoplastic left heart syndrome, aortic coarctation, and atrioventricular canal defects were commonly associated with ASA.

Progression of Tricuspid Regurgitation After Repaired Functional Ischemic Mitral Regurgitation

Despite correction of left-sided cardiac lesions, associated functional tricuspid regurgitation (TR) that was surgically ignored can persist. It can also appear de novo. The aim of this study was to analyze TR in a group of patients who underwent successful revascularization and mitral valve repair (MVRep) for functional ischemic mitral regurgitation (MR). Among 124 consecutive patients with MVRep, 70 left the operating room with MR < or =1+ and had a preoperative and follow-up transthoracic echocardiograph. Moderate or greater MR or TR was considered significant. Twenty-one patients (30%) had TR before surgery, and only 9 had TR repaired. The postoperative incidence of residual TR was not significantly different whether the tricuspid valve had been repaired (4 of 9 [44%]) or surgically ignored (8 of 12 [67%]). At last follow-up, 34 patients (49%) had significant TR. The incidence of TR increased from 25% at <1 year to 53% between 1 and 3 years and 74% at >3 years. Absence or presence of recurrent MR did not significantly affect TR (14 of 22 [64%] with MR versus 20 of 48 [42%] with no MR). Preoperative and postoperative tricuspid annulus size in patients with late TR was significantly larger than in patients without TR. Functional TR is frequently associated with functional ischemic MR. After MVRep, close to 50% of patients have TR. The incidence of postoperative TR increases with time. Preoperative tricuspid annulus dilation might be a predictor of late TR.

Left Heart Lesions in Patients With Ebstein Anomaly

To identify the incidence of left heart abnormalities in patients with Ebstein anomaly, recognizing that left-sided lesions in this patient group have been overlooked. According to the echocardiography database at the Mayo Clinic in Rochester, Minn, 106 consecutive patients with Ebstein anomaly underwent echocardiography between July 1, 2001, and February 28, 2003. Clinical data as well as electrocardiographic and echocardiographic reports and images were reviewed. Ebstein anomaly was severe in 76 patients (72%). Previous tricuspid valve surgery was reported in 46 patients (43%), and previous closure of an atrial septal defect or patent foramen ovale was reported in 34 patients (32%). Left ventricular (LV) myocardial changes resembling noncompaction occurred in 19 patients (17.9%), LV systolic dysfunction in 7 patients (7%), LV diastolic dysfunction in 34 (36%) of 95 patients, and LV dilatation in 4 patients (4%). Additional left-sided cardiac lesions included mitral valve prolapse in 16 patients (15%), bicuspid aortic valve in 8 (8%), mitral valve dysplasia in 4 (4%), and ventricular septal defect in 8 (8%). Wolff-Parkinson-White syndrome occurred in 22 patients (21%). The QRS axis tended to be different in LV noncompaction with a mean +/- SD axis of 12 degrees +/- 74 degrees vs 36 degrees +/- 66 degrees overall (P=.08). Otherwise, there were no differences in clinical or surgical data between the groups with normal and abnormal LV myocardium. In patients with Ebstein anomaly, left heart abnormalities involving the myocardium or valves were observed in 39% of patients. Ebstein anomaly should not be regarded as a disease confined to the right side of the heart.