Posterior pericardial ascending-to-descending aortic bypass: an alternative surgical approach for complex coarctation of the aorta.

BACKGROUND: Coarctation of the aorta is commonly associated with recoarctation or additional cardiovascular disorders that require intervention. The best surgical approach in such patients is uncertain. Ascending-to-descending aortic bypass graft via the posterior pericardium (CoA bypass) allows simultaneous intracardiac repair or an alternative approach for the patient with complex coarctation. METHODS AND RESULTS: Between 1985 and 2000, 18 patients (13 males and 5 females, mean age 43+/-13 years) with coarctation of the aorta underwent CoA bypass through median sternotomy. Before operation, average New York Heart Association class was II (range I to IV), and 15 patients (83%) had systemic hypertension. One or more previous cardiovascular operations had been performed in 12 patients (67%); 10 patients had >/=1 prior coarctation repair. Two patients had prior noncoarctation cardiovascular surgery. Concomitant procedures performed in 14 patients (78%) included the following: aortic valve replacement in 9; coronary artery bypass surgery in 3; mitral valve repair in 2; and septal myectomy, mitral valve replacement, aortoplasty, subaortic stenosis resection, ventricular septal defect closure, and ascending aorta replacement in 1 patient each. All patients survived the operation and were alive with patent CoA bypass at a mean follow-up of 45 months. No graft-related complications occurred, and there were no instances of stroke or paraplegia. Systolic blood pressure fell from 159 mm Hg before surgery to 125 mm Hg after surgery. CONCLUSIONS: CoA bypass via median sternotomy can be performed with low morbidity and mortality. Although management must be individualized, extra-anatomic CoA bypass via the posterior pericardium is an excellent single-stage approach for patients with complex coarctation or recoarctation and concomitant cardiovascular disorders.

Ebstein's anomaly: outcome of pregnancy.

OBJECTIVES: The outcome of pregnancy in Ebstein's anomaly was studied in 72 such patients (44 women, 28 men) who had had pregnancies or fathered children. BACKGROUND: Patients with Ebstein's anomaly often reach childbearing age. Reports of the outcome of pregnancy in Ebstein's anomaly are available; however, the number of patients is small. METHODS: The medical and surgical data bases at the Mayo Clinic were reviewed, and 145 patients (62 men, 83 women) of childbearing age with Ebstein's anomaly were located. All patients were contacted, and 72 patients (44 women, 28 men) with offspring were identified and reviewed in detail to assess the outcome of pregnancy. RESULTS: Forty-four women had 111 pregnancies resulting in 85 live births (76%). Seventy-six deliveries (89%) were vaginal, and nine (11%) were by cesarean section. Twenty-three deliveries were premature. There were 19 spontaneously unsuccessful pregnancies, 7 therapeutic abortions and 2 early neonatal deaths. The mean birth weight of the infants born to cyanotic women was 2.53 kg, which was significantly lower than the mean birth weight of infants born to acyanotic women (3.14 kg [p < 0.001]). The overall incidence of congenital heart disease in the 158 offspring of parents with Ebstein's anomaly was 4% (6 of 158). The incidence of congenital heart disease was 6% (5 of 83) in the offspring of women with Ebstein's anomaly and 1% (1 of 75) in that of men. There was a 0.6% (1 of 158) incidence of familial Ebstein's anomaly. There were no serious pregnancy-related maternal complications, which included maternal death, stroke, congestive heart failure, arrhythmias or endocarditis. CONCLUSIONS: Pregnancy in women with Ebstein's anomaly is well tolerated. It is associated with an increased risk of prematurity, fetal loss and congenital heart disease in the offspring. In addition, a significantly lower birth weight is found in the offspring of cyanotic versus acyanotic women with Ebstein's anomaly. Paternal Ebstein's anomaly also seems to result in an increased risk of congenital heart disease in the offspring compared with the incidence in the general population.

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.

Pregnancy among women with congenitally corrected transposition of great arteries.

OBJECTIVES: The outcome of pregnancy in congenitally corrected transposition of the great vessels was studied in 22 women. BACKGROUND: Women with congenitally corrected transposition of the great vessels often reach childbearing age. Although reports on the outcome of pregnancy in these women are available, the number of patients is small. METHODS: The medical and surgical databases at the Mayo Clinic were reviewed, and 36 women >16 years old with congenitally corrected transposition of the great vessels were identified. All of them were contacted, and 22 who had pregnancies were identified and the outcome of pregnancy was evaluated. RESULTS: Twenty-two women had 60 pregnancies resulting in 50 live births (83%). Forty-four deliveries (88%) were vaginal and 6 (12%) were by cesarean section. One delivery was premature at 29 weeks. There was one successful twin pregnancy. There were 11 unsuccessful pregnancies. One patient developed congestive heart failure late in pregnancy because of systemic atrioventricular valve regurgitation and required valve replacement in the early postpartum period. One patient had a total of 12 pregnancies, including 1 twin pregnancy and 2 unsuccessful pregnancies. She had multiple pregnancy-related complications, including toxemia, congestive heart failure, endocarditis and myocardial infarction (single coronary artery). No other serious pregnancy-related maternal complications and no pregnancy-related deaths occurred. The mean birth weight of the infants (n = 32) was 3.2 +/- 0.4 kg. None of the 50 live offspring have been diagnosed with congenital heart disease. CONCLUSIONS: Successful pregnancy can be achieved in most women with congenitally corrected transposition of the great arteries. The rate of fetal loss and maternal cardiovascular morbidity is increased. Because of the small number of births, the risk of congenital heart disease in offspring of women with congenitally corrected transposition of the great arteries is uncertain.

Coarctation of the aorta: outcome of pregnancy.

OBJECTIVES: The study sought to determine the outcome of pregnancy in women with coarctation of the thoracic aorta. BACKGROUND: Patients with coarctation of the thoracic aorta are expected to reach childbearing age, but data on the outcome of pregnancy in this population are limited. METHODS: The Mayo Clinic database was reviewed for women of childbearing age (> or =16 years old) with a diagnosis of aortic coarctation evaluated from 1980 to 2000. Spectrum of cardiovascular disease, surgical history, and obstetrical and neonatal outcomes were determined. RESULTS: Fifty women with coarctation had pregnancies: 30 had coarctation repair before pregnancy, 10 had repair after pregnancy, 4 had repair both before and after pregnancy, and 6 had no history of repair. The 50 women had 118 pregnancies resulting in 106 births. There were 11 miscarriages (9%), 4 premature deliveries (3%), and 1 early neonatal death; 38 deliveries (36%) were by cesarean section. Of the 109 offspring, 4 (4%) had congenital heart disease. A patient with Turner syndrome died of a Stanford type A dissection at 36 weeks of pregnancy. Nineteen women (38%) were known to have hemodynamically significant coarctation during pregnancy (gradient > or =20 mm Hg). Fifteen women (30%) had hypertension during their pregnancy, 11 of whom (73%) had hemodynamically significant coarctation during that time (8 with native and 3 with residual/recurrent coarctation). CONCLUSIONS: Major cardiovascular complications were infrequent but continue to be a source of concern for patients with coarctation who become pregnant. Systemic hypertension during pregnancy was common and related to the presence of a significant coarctation gradient.

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.

Noncardiac surgery in Eisenmenger syndrome.

OBJECTIVES: The purpose of this study was to evaluate the morbidity and mortality associated with noncardiac surgery (NCS) in patients (pts) with Eisenmenger syndrome. BACKGROUND: Noncardiac surgery in pts with Eisenmenger syndrome is associated with increased cardiovascular complications. METHODS: Fifty-eight pts with Eisenmenger syndrome (17M, 41F aged 18 to 69 years (mean 41 years) who had been followed for up to 41.5 years (mean 9.3 years) were retrospectively evaluated for any NCS done at > or = 17 years of age. RESULTS: Twenty-four pts had a total of 28 NCSs at an age of 17 to 55 years (mean 29 years) including 9 tubal ligations, 3 neurosurgeries, 3 cholecystectomies, 3 hysterectomies, 3 vasectomies, and 1 each spinal fusion, appendectomy, eye enucleation, hernia repair, hand surgery, tonsillectomy and therapeutic abortion. There were two deaths (7%), one following spinal fusion and the other following appendectomy at another institution. Fourteen of these NCSs were performed at our institution, including 11 under general anesthesia. The duration of anesthesia varied from 75 to 525 min (mean 165 min). All pts remained in sinus rhythm. The lowest systolic blood pressure (BP) ranged from 78 to 125 mm Hg. Of those 11 pts, 9 were extubated immediately after surgery and 2 needed dopamine. Ten patients were discharged without any complications, including 3 within 1 day of surgery. One death occurred 10 days following spinal fusion. This pt had the longest anesthesia (525 min) and an intraoperative systolic BP as low as 78 mm Hg. She also needed the largest fluid administration (6,475 cc) in addition to postoperative mechanical ventilation and dopamine. CONCLUSIONS: Adult pts with Eisenmenger syndrome are at increased risk with NCS, but with current/modern techniques, the risk of death is less than previously thought. In the vast majority of cases, NCS can be undertaken without substantial morbidity, and early extubation is achievable. However, even with relatively minor surgery, significant complications, including death, can occur. Referral to major centers with expertise in the care of pts with Eisenmenger syndrome is advisable.

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.

Intravascular ultrasound imaging: in vitro validation and pathologic correlation.

Intravascular ultrasound imaging is a new method in which high resolution images of the arterial wall are obtained with use of a catheter placed within an artery. An in vitro Plexiglas well model was used to validate measurements of the luminal area, and an excellent correlation was obtained. One hundred thirty segments of fresh peripheral arteries underwent ultrasound imaging and the findings were compared with the corresponding histopathologic sections. Luminal areas determined with ultrasound imaging correlated well with those calculated from microscopic slides (r = 0.98). Three patterns were identified on the ultrasound images: 1) distinct interface between media and adventitia, 2) indistinct interface between media and adventitia but different echo density layers, and 3) diffuse homogeneous appearance. The types of patterns depended on the relative composition of the media and adventitia. Calcification of intimal plaque obscured underlying structures. Atherosclerotic plaque was readily visualized but could not always be differentiated from the underlying media.

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.

The heart in massive (more than 300 pounds or 136 kilograms) obesity: analysis of 12 patients studied at necropsy.

Observations are described in 12 massively obese patients (5 women, 7 men), aged 25 to 59 years (mean 37), who weighed 312 to more than 500 pounds (mean 381). Seven patients had had systemic hypertension, 4 hypersomnia or sleep apnea, 2 diabetes mellitus, and 1 patient symptomatic coronary artery disease. Five patients died suddenly from undetermined causes, 2 from right-sided congestive heart failure, 1 patient from acute myocardial infarction; 1 from aortic dissection; 1 from intracerebral hemorrhage; 1 from a drug overdose, and 1 soon after an ileal bypass. The heart weight was increased in all 12 patients. The heart weight to body weight ratio expressed as a percent ranged from 0.22 to 0.61 (mean 0.37) (normal for men 0.42 to 0.46 [mean 0.43], normal for women 0.38 to 0.46 [mean 0.40]). The left ventricular cavity was dilated in 11 patients and the right ventricular cavity in all 12. Only 2 patients (aged 42 and 59 years) had 1 or more major epicardial coronary arteries narrowed greater than 75% in cross-sectional area by atherosclerotic plaque, 1 of whom had no symptoms of myocardial ischemia. Of 664 five-millimeter segments from the 4 major epicardial coronary arteries from 11 patients (mean 60 per patient), 431 (65%) were narrowed 0 to 25% in XSA, 143 (21%) were narrowed 26 to 50%, 73 (11%) were narrowed 51 to 75%, and 17 (3%) were narrowed 76 to 100%.(ABSTRACT TRUNCATED AT 250 WORDS)

Sudden coronary death: comparison of patients with to those without coronary thrombus at necropsy.

Among 70 victims of sudden coronary death (SCD), certain clinical and morphologic findings in the 13 with a coronary thrombus are compared with the findings in 57 victims without a coronary thrombus. The 13 with a thrombus were younger than those without (mean age 43 vs 51 years, p less than 0.02); had a lower mean percent of cross-sectional area (XSA) narrowing by plaque at the site of maximal coronary stenosis (89% vs 95%, p less than 0.01); and had a higher mean percent of 5-mm segments of the 4 major epicardial coronary arteries minimally narrowed (0 to 25% in XSA) by plaque (27% vs 19%, p less than 0.001). No differences occurred in the 2 groups with regard to sex, previous angina pectoris or clinical acute myocardial infarction, healed myocardial infarction at necropsy, mean heart weight, number of major coronary arteries narrowed 76 to 100% in XSA by atherosclerotic plaque, or the mean percent of 5-mm segments of the 4 major epicardial coronary arteries narrowed 76 to 100% in XSA by atherosclerotic plaque. Thus, coronary thrombi are infrequent in victims of SCD, and when observed, their significance is uncertain because victims of SCD without coronary thrombi have similar amounts of severe coronary narrowing.

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.

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.

Sudden coronary death: relation of amount and distribution of coronary narrowing at necropsy to previous symptoms of myocardial ischemia, left ventricular scarring and heart weight.

The amount and distribution of coronary arterial narrowing by atherosclerotic plaque at necropsy is described in 70 victims, aged 22 to 81 years (mean 50), of sudden coronary death. Of 3,484 five-millimeter segments examined (mean 50 per patient) from the 4 major (left main, left anterior descending, left circumflex and right) coronary arteries, 950 (27%) were narrowed 76 to 100% in cross-sectional area (XSA), 1,127 (32%), 51 to 75%; 689 (20%), 26 to 50%; and 718 (21%), 0 to 25%. More extensive severe narrowing occurred in the proximal than in the distal halves of the left anterior descending, left circumflex and right coronary arteries. Comparison between the 31 previously symptomatic victims (angina pectoris or a clinical acute myocardial infarction or both) with the 39 victims who had previously been asymptomatic disclosed a significantly higher mean percent of severely narrowed (76 to 100% XSA) 5-mm segments (30 vs 25%, p less than 0.005) and lower mean percent of minimally narrowed (0 to 25% XSA) segments in the symptomatic group (15 vs 25%, p less than 0.001). Comparison of the 31 patients who had a healed myocardial infarction at necropsy with the 39 patients who did not disclosed a higher mean percent of 5-mm segments narrowed 76 to 100% in XSA (33 vs 24%, p less than 0.001) and a lower mean percent of segments narrowed minimally in those with a left ventricular scar (13 vs 26%, p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Wear of the metallic studs on the composite seat of the 2320 Starr-Edwards aortic valve and its clinical consequences.

The 2320 Starr-Edwards aortic valve has a composite seat with metallic studs that protrude through the cloth. Certain clinical and morphologic findings are described in 6 patients who had this prosthesis in situ for 50 to 144 months (mean 92). Considerable disruption of the cloth covering both struts and base, and varying degrees of wear of the metallic studs that line the luminal side of the prosthetic ring occurred in each. Excessive stud wear resulted in severe disruption of the cloth lining the interior of the prosthetic ring. Cloth disruption may be associated with hemolytic anemia, embolic consequences, or both. Of our 6 patients, 4 had severe hemolytic anemia, 4 had neurologic events compatible with emboli and 1 died suddenly. Stud wear as observed in the 2320 series Starr-Edwards prosthesis also may occur in the models 2310 and 2400 prostheses, which have the same composite seat.

Effect of elevating the wrist on the radial pulse in aortic regurgitation: Corrigan revisited.

Five patients with severe aortic regurgitation had their intraarterial radial pulse recorded simultaneously with the external radial artery deflection of the other wrist. Recordings were made with the arms horizontal and repeated after elevation of the wrists, until the arms were vertical. Elevating the arm of a patient with aortic regurgitation increased the pulsation recorded over the radial artery, lowered mean intraarterial pressure, and reduced the pulse pressure. The results suggest that elevation increases the compliance of the arterial wall, primarily by moving the artery to a lower position on its pressure-volume curve. The same changes occurred in 5 normal subjects but were less pronounced.

Comparison of late degenerative changes in porcine bioprostheses in the mitral and aortic valve position in the same patient.

Porcine bioprostheses implanted in both the mitral and aortic valve positions simultaneously in 5 patients aged 20 to 61 years (mean 45) were reexamined 18 to 107 months (mean 51) later. In 4 patients, the degenerative changes were distinctly more severe in the bioprostheses in the mitral than in the aortic valve position.

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.

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.