[Canadian Consensus Conference on Congenital Heart Defects in the Adult 1996].
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Adult↗
Biomedical subjects
C A Warnes
Publications and source records attributed to C A Warnes.
Outcome of pregnancy in patients with complex pulmonic valve atresia.
Fifteen women with complex pulmonary atresia who had pregnancies were retrospectively reviewed. Although no pregnancy-related deaths occurred, complications were noted in 3 patients and risk of fetal loss and premature birth were increased, none of the 10 offspring had congenital heart disease.
Abortion, Spontaneous↗
Mimics of Ebstein's anomaly.
The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.
Adolescent↗
Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.
OBJECTIVE: Little attention has been paid to the occurrence of aortic regurgitation after complete repair in patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot. To highlight the development of aortic regurgitation or aortic root dilation severe enough to necessitate aortic valve replacement with or without aortic aneurysmorrhaphy or aortic root replacement, we retrospectively reviewed the records of patients who underwent aortic valve operation at our institution subsequent to repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. METHODS: We searched the Mayo Clinic database for patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot who subsequently had aortic valve or aortic root operations. The degree of aortic regurgitation before operation was noted. Aortic sinus and root dimensions were measured. RESULTS: Sixteen patients underwent complete repair at a median age of 17 years, followed by an aortic operation a median of 13.5 years later. All 16 patients had dilated aortic sinuses at the time of the aortic valve operation. These 16 patients had aortic valve replacement: 11 with mechanical prostheses and 5 with bioprostheses. Five of the 16 also had reduction of aortic dilation by lateral aneurysmorrhaphy, and 1 had graft replacement of the ascending aorta. Five patients had associated conditions (evidence of valvular damage, recurrent ventricular septal defect, or history of endocarditis) discovered at the aortic valve operation that have been reported to be related to the development of aortic regurgitation. The remaining 11 patients had progressive aortic regurgitation despite complete, uncomplicated repair. CONCLUSIONS: Progressive aortic regurgitation and aortic root dilation can occur despite complete repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.
Adolescent↗
Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography.
OBJECTIVE: This study sought to demonstrate that with proper technique, identification of the normal and abnormal pulmonary venous connection can be made with confidence using transesophageal echocardiography (TEE). BACKGROUND: Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital anomaly whose diagnosis has classically been made using angiography. METHODS: We performed a retrospective review of all patients of all ages with PAPVC diagnosed at the Mayo Clinic who had undergone TEE because of either right ventricular volume overload or suspected intracardiac shunting by transthoracic echocardiography or intraoperatively. RESULTS: A total of 66 PAPVCs were detected in 43 patients (1.5/patient); in 2 additional patients, TEE suggested, but did not diagnose, PAPVCs. Shortness of breath was the most common presenting symptom (42.2%), followed by heart murmur and supraventricular tachycardia. Right-sided anomalous veins were identified in 35 patients (81.4%), left-sided in 7 (16.3%) and bilateral in 1 (2.3%). There was a single anomalous connecting vein in 23 patients (53.5%), two in 18 (41.9%), three in 1 (2.3%) and four in 1 (2.3%). The connecting site was the superior vena cava (SVC) in 39 veins (59.1%), right atrial-SVC junction in 6 (9.1%), right atrium in 8 (12.1%), inferior vena cava in 1 (1.5%) and the coronary sinus in 2 (3.0%). Ten anomalous left pulmonary veins were connected by a vertical vein to the innominate vein (15.1%). Sinus venosus atrial septal defect (ASD) was the most common associated anomaly in 22 patients (49%), followed by ostium secundum ASD in 6 and patent foramen ovale in 4. Fifteen patients had an intact atrial septum. Thirty-one patients (68.8%) underwent surgical repair. PAPVC was confirmed in all patients, including the two whose TEE results were suggestive of PAPVC. All 49 PAPVCs detected by TEE preoperatively were confirmed at the time of operation. CONCLUSIONS: TEE is highly diagnostic for PAPVC and can obviate angiography. Accurate anatomic diagnosis may influence the need for medical and surgical management. TEE should be performed in patients with right ventricular volume overload when the precordial examination is inconclusive.
Adult↗
Permanent pacing in Ebstein's anomaly.
Patients with Ebstein's anomaly present unique challenges to permanent pacing due to anatomical variations and tricuspid valve replacement. We retrospectively reviewed our experience with permanent pacing in patients with Ebstein's anomaly between 1976 and 1993. We identified 401 patients with Ebstein's anomaly, of whom 15 (3.7%) required permanent pacing (1 of the 15 was implanted elsewhere). Of the 15, there were 8 females and 7 males (mean age 32 years [range 7-74]); the indications for pacing were AV block in 11 and sinus node dysfunction in 4. Eight patients were programmed with VVI and seven with DDD. All VVI patients were paced epicardially. Two patients with DDD pacemakers had transvenous atrial and ventricular leads, 4 DDD patients had transvenous atrial leads and epicardial ventricular leads, and 1 patient had both epicardial and transvenous systems. Associated surgical procedures included tricuspid valve replacement in 14 of 15, atrial septal defect repair in 10 of 15, atrioplasty in 7 of 15, prior tricuspid annuloplasty in 4 of 15, pulmonary vein dilation in 1 of 15, and conduction system ablation in 2 of 15. Patients had a mean follow-up of 35 months (range 1-168 months). Complications requiring operative intervention occurred in four patients. One patient had displacement of a transvenous ventricular lead. A second patient had an epicardial lead failure. A third patient had a nonfunctioning atrial lead that displaced across the tricuspid valve, causing severe tricuspid regurgitation. The fourth patient had multiple epicardial and endocardial leads exit block with secondary diaphragmatic stimulation. Permanent pacemakers were required in 3.7% of patients with Ebstein's anomaly, with the indication being intrinsic conduction disease in the majority of patients. Ninety-three percent of patients required tricuspid valve replacement, suggesting more severe manifestation of Ebstein's anomaly. Twenty-seven percent had complications requiring surgical intervention. Thus, permanent pacing in patients with Ebstein's anomaly can be challenging and should be approached by an experienced physician.
Adolescent↗
Coarctation of the aorta: collateral flow assessment with phase-contrast MR angiography.
OBJECTIVE: The purpose of this report is to describe a new use of MR imaging in coarctation of the aorta. The specific question addressed was how well collateral blood flow in intercostal arteries, as determined by phase-contrast MR angiography, correlated with findings during surgery or catheterization in patients with coarctation of the aorta. CONCLUSION: Phase-contrast MR angiography is an excellent technique for detecting the presence or absence of collateral blood flow in the intercostal arteries of patients with coarctation of the aorta. Knowing whether collateral blood flow is present in patients with narrowing of the juxtaductal aorta should help assess the clinical hemodynamic significance of the coarctation.
Adult↗
Diagnosis of sinus venosus atrial septal defect with transesophageal echocardiography.
BACKGROUND: Sinus venosus atrial septal defect (SVD) is underdiagnosed with transthoracic echocardiography because of its posterior (far field) location. Transesophageal echocardiography (TEE) should be ideally suited to diagnose SVD, given the proximity of the transducer to the defect. METHODS AND RESULTS: A retrospective study was undertaken that used the medical history, echocardiographic findings, and surgical data of patients identified from computer records as having the diagnosis of SVD during the period in which TEE has been in use (1987 to 1995). Twenty-five patients (14 females and 11 males; median age, 45 years; range, 10 to 75 years) with SVD had TEE between 1987 and 1995. Prior transthoracic echocardiography clearly defined the SVD in 3 of these patients, and it was suspected in another 11 on the basis of color-flow imaging. Ten patients had unexplained dilatation of the right side of the heart, which prompted TEE examination. SVD was visualized with TEE in all 25 patients and ranged in size from 1 to 3 cm. Thirty-seven right-sided anomalous pulmonary venous connections were identified in 23 patients. No left-sided anomalous pulmonary venous connections were detected. Anatomic confirmation was obtained in all 23 surgical patients. No patient required preoperative cardiac catheterization for diagnosis. CONCLUSIONS: TEE is accurate for the diagnosis of SVD and should be undertaken in any patient with unexplained dilatation of the right side of the heart. The associated pulmonary venous abnormalities can be identified with TEE. Cardiac catheterization for diagnostic purposes should not be required before surgical correction.
Adolescent↗
Survival into adulthood of patients with unoperated single ventricle.
Adult patients with double-inlet left ventricle and perfectly balanced circulation may survive into the sixth decade with good functional capacity and preserved ventricular function. This should be considered before such patients are referred for a Fontan repair.
Adult↗
Congenitally corrected transposition: the uncorrected misnomer.
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Humans↗
Improved early morbidity and mortality after Fontan operation: the Mayo Clinic experience, 1987 to 1992.
OBJECTIVES: This study sought to evaluate changes in early morbidity and mortality as well as predictors of outcome in our most recent 339 patients undergoing modified Fontan operations. BACKGROUND: The Fontan operation is the preferred definitive palliation for patients with functional single ventricles. Previously reported early mortality rates after Fontan operation have been substantial. METHODS: Records of 339 consecutive patients who had a Fontan operation at the Mayo Clinic between 1987 and 1992 (recent cohort) were reviewed. This cohort was compared with the previous 500 patients who had Fontan operations performed between 1973 and 1986 (early cohort). RESULTS: Recently, overall early mortality after Fontan has decreased significantly compared with that for the early cohort (from 16% to 9%, p = 0.002). This decline occurred despite increased anatomic complexity of patients. Short-term posthospital survival has also improved significantly in recent patients. One-year survival improved to 88% from 79%, and 5-year survival to 81% from 73% (p = 0.006). Patients with common atrioventricular valves and those who took daily preoperative diuretic medication or had either postoperative renal failure or elevated postbypass right atrial pressure were at increased risk for early mortality. Young age was not found to be a risk factor for early mortality. Early mortality for patients with heterotaxia decreased dramatically: recent 30-day mortality was 15% compared with 41% in the early heterotaxy cohort. CONCLUSIONS: Many factors may have contributed to decreased early mortality after Fontan. Improved patient selection, younger age at time of operation, refinements in surgical techniques and postoperative management may all have had important roles. Proposed technical modifications of the Fontan operation must be evaluated in light of these improved results.
Age Factors↗
Cerebrovascular events in adult patients with cyanotic congenital heart disease.
OBJECTIVES: We sought to determine the frequency of spontaneous cerebrovascular events in adult patients with cyanotic congenital heart disease and to evaluate any contributing factors. BACKGROUND: Cerebrovascular events are a serious complication of cyanotic congenital heart disease in infants and children but are said to be uncommon in adults. METHODS: Between 1988 and 1995, 162 patients with cyanotic congenital heart disease (mean age 37 years, range 19 to 70) were retrospectively evaluated for any well documented cerebrovascular events that occurred at > or = 18 years of age. Events related to procedures, endocarditis or brain abscess were excluded. RESULTS: Twenty-two patients (13.6%) had 29 cerebrovascular events (1/100 patient-years). There was no significant difference between those with and without a cerebrovascular event in terms of age, smoking history, degree of erythrocytosis, ejection fraction or use of aspirin or warfarin (Coumadin). Patients who had a cerebrovascular event had a significantly increased tendency to develop hypertension, atrial fibrillation, microcytosis (mean corpuscular volume < 82) and history of phlebotomy (p < 0.05). Even when patients with hypertension or atrial fibrillation were excluded, there was an increased risk of cerebrovascular events associated with microcytosis (p < 0.01). CONCLUSIONS: Adults with cyanotic congenital heart disease are at risk of having cerebrovascular events. This risk is increased in the presence of hypertension, atrial fibrillation, history of phlebotomy and microcytosis, the latter condition having the strongest significance (p < 0.005). This finding leads us to endorse a more conservative approach toward phlebotomy and a more aggressive approach toward treating microcytosis in adults with cyanotic congenital heart disease.
Adult↗
Protein-losing enteropathy after the Fontan operation: successful treatment by percutaneous fenestration of the atrial septum.
Protein-losing enteropathy (PLE) after the Fontan operation is a life-threatening complication that may be refractory to medical therapy. Herein we describe a percutaneous atrial fenestration that was performed in a 42-year-old man with a double-inlet left ventricle who had undergone a Fontan operation 9 years earlier. Severe PLE developed, and despite frequent infusions of protein, his albumin level was 1.8 g/dL. The diagnosis of PLE was confirmed by an alpha(1)-antitrypsin clearance of 425 mL in 24 hours (normal 27 or less). Percutaneous atrial fenestration resulted in dramatic clinical improvement and resolution of the PLE. At 5-month follow-up, the patient's albumin level was 4.2 g/dL, his alpha(1)-antitrypsin clearance was normal, and he was free of ascites and edema.
Adult↗
Partial atrioventricular canal defect: long-term follow-up after initial repair in patients > or = 40 years old.
OBJECTIVES: This study was undertaken to determine the results of repair of partial atrioventricular (AV) canal in patients > or = 40 years old. BACKGROUND: Although postoperative outcomes in younger patients have been well documented, the fate of older patients with repaired partial AV canal is less clear. METHODS: From 1958 to 1990, 31 patients 40 to 71 years old (mean age 51) had repair of partial AV canal. Twenty-three patients had repair of the cleft mitral valve; two had mitral valve replacements; and six needed no mitral valve operation. RESULTS: Early mortality was 6%. One patient was lost to follow-up. Nine of the early survivors are known to have died. There is a small but significant development over the long term of atrial arrhythmias, complete heart block, subaortic stenosis, recurrent mitral regurgitation and, rarely, mitral stenosis. Three of the 28 patients available for follow-up had mitral valve reoperation and subaortic stenosis developed in 2. Nineteen patients were alive in 1991 (mean follow-up 14 years). Seven patients were in New York Heart Association functional class I, eight were in class II, and four were in class III. Fifteen of the 19 patients reported sustained postoperative improvement. CONCLUSIONS: Patients > or = 40 years old can have partial AV canal repair with low risk. Long-term survival is good, with subjective improvement in symptoms. Late complications occur but are uncommon, suggesting that long-term follow-up is warranted.
Adult↗
Guidelines for training in adult cardiovascular medicine. Core Cardiology Training Symposium (COCATS). Task Force 9: training in the care of adult patients with congenital heart disease.
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Adult↗
Results of an autologous tissue reconstruction for replacement of obstructed extracardiac conduits.
Between May 1983 and March 1, 1995, 50 patients had replacement of an obstructed pulmonary ventricle-pulmonary artery conduit with an autologous tissue reconstruction in which a prosthetic roof was placed over the fibrous tissue bed of the explanted conduit. The roof was constructed with xenograft pericardium (most recently) (n = 42), homograft dura mater (n = 5), or Dacron fabric (n = 3). Patient ages ranged from 5 to 34 years (median 16 years). The explanted conduits were Hancock conduits (n = 33), Tascon conduits (n = 6), homograft (n = 4), Dacron tube (n = 3), and others (n = 4). Preoperative maximum systolic gradients ranged from 44 to 144 mm Hg (median 78 mm Hg). Thirty-seven concomitant cardiac procedures were done in 29 patients. When a valve was necessary (n = 15), it was possible to place a large-sized valve in the autologous tissue reconstructions (range 22 to 29 mm, median 26 mm). Cardiopulmonary bypass times ranged from 34 to 223 minutes (median 84 minutes), and aortic crossclamp times ranged from 0 (in 32 patients) to 109 minutes (median 0 minutes). Intraoperative postrepair peak systolic gradients from pulmonary ventricle to pulmonary artery ranged from 0 to 33 mm Hg (median 13 mm Hg). There was one early death (2%) in a patient who had additional cardiac procedures. Follow-up was complete in all patients and ranged from 1 month to 11.8 years (median 7.5 years). There were two sudden late deaths: conduits in both were known to be free from obstruction. Forty-four of the 47 surviving patients had evaluation of the gradient by echocardiography or cardiac catheterization 1 month to 11 years (median 7 years) after operation. The gradients ranged from 5 to 45 mm Hg (median 20 mm Hg). None of the conduits developed an obstructive peel, valve obstruction, or valve incompetence. At 10 years, the freedom from reoperation for conduit obstruction was 100%, and freedom from reoperation for any cause was 81%. This technique simplifies conduit replacement, allows for a generous-sized outflow tract, has a low risk, and yields late results that appear superior to those of cryopreserved homografts or other types of extracardiac conduits.
Adolescent↗
Late results of systemic atrioventricular valve replacement in corrected transposition.
From December 1964 to October 1993, 40 patients (aged 5 months to 70 years, mean 21.8 years, median 13.6 years) with corrected transposition and systemic atrioventricular valve insufficiency underwent replacement (n = 39) or repair (n = 1) of the systemic atrioventricular valve. Thirty-nine patients had situs solitus and 1 had situs inversus. Associated anomalies included Ebstein's malformation of the systemic atrioventricular valve (n = 22), ventricular septal defect (n = 19), and pulmonary stenosis (n = 14). Preoperatively, 16 patients (40.0%) had complete heart block and 27 patients (67.5%) were in New York Heart Association functional classes III and IV. The early mortality was 10.0% (n = 4) and 8 patients died subsequently. The principal cause of death in all 12 patients was systemic ventricular failure. Overall survival including early mortality was 78.0% at 5 years and 60.7% at 10 years; survival excluding early mortality was 86.7% at 5 years and 67.5% at 10 years. Survivorship correlated with preoperative systemic ventricular ejection fraction of 44% or more (p < 0.001) and later interval of operation (9 deaths in 15 patients before 1981 versus 3 deaths in 25 patients subsequently) (p = 0.06). There were no cases of surgically induced complete heart block. Two patients underwent late reoperations related to the systemic atrioventricular valve prosthesis. Follow-up extended to 26.0 years (median 4.7 years). At last follow-up, 18 of the 28 survivors were in New York Heart Association functional class I, 9 were in class II, and 1 was in class III. We conclude that the results of systemic atrioventricular valve replacement in corrected transposition have improved significantly during the past decade. To preserve systemic ventricular function, we suggest operation be considered at the earliest sign of progressive ventricular dysfunction as assessed by serial clinical evaluation and echocardiography.
Adolescent↗
Adults with congenital heart disease: patient knowledge of endocarditis prophylaxis.
OBJECTIVE: To determine whether adults with congenital heart disease have adequate knowledge of infective endocarditis and endocarditis prophylaxis and to ascertain whether an educational program effectively improves patient knowledge and compliance. MATERIAL AND METHODS: We asked 102 consecutive patients to complete a 12-question survey to assess their knowledge of heart disease, infective endocarditis, and endocarditis prophylaxis. RESULTS: Of 102 patients, 100 (98%) completed the questionnaire. Sixty-eight patients knew the name of their heart disease. Fifty patients correctly defined endocarditis, but only 43 knew hygiene measures that could prevent endocarditis. Ninety-six patients knew that they needed to take "a medicine" before dental procedures, and 76 of those patients (79%) knew that an antibiotic was necessary. Patient use of cardiac medications and a history of endocarditis correlated significantly with knowledge of endocarditis. Patients who had been to the Adult Congenital Heart Disease Clinic at least once knew endocarditis prevention measures and the importance of regular dental and cardiology follow-up significantly more frequently than did first-time attendees. Despite educational counseling, however, patient recall of endocarditis and its prevention is disappointing. CONCLUSION: Many adults with congenital heart disease have inadequate knowledge of their cardiac lesion, endocarditis, and endocarditis prophylaxis. Educational efforts for adults with congenital heart disease need to be updated and reinforced regularly.
Adolescent↗