Echocardiographic Strain to Predict Need for Transplant or Mortality in Fontan Patients with Hypoplastic Left Heart Syndrome.

Despite recent advances, hypoplastic left heart syndrome (HLHS) patients subsequent to the Fontan still have significant morbidity and mortality. Some require heart transplant due to systemic ventricular dysfunction. Limited data exist on timing for transplant referral. This study aims to correlate systemic ventricular strain by echocardiography to transplant-free survival. HLHS patients who had Fontan palliation at our institution were included. Patients were divided into: 1) Required transplant or experienced mortality (composite end point); 2) Did not require transplant or survived. For those who experienced the composite endpoint, the last echocardiogram prior to the composite outcome was used, while for those who did not experience the composite endpoint the last echocardiogram obtained was used. Several qualitative and quantitative parameters were analyzed with focus on strain parameters. Ninety-five patients with HLHS Fontan palliation were identified. Sixty-six had adequate images and eight (12%) experienced transplant or mortality. These patients had greater myocardial performance index by flow Doppler (0.72 versus 0.53, p = 0.01), higher systolic/diastolic duration ratio (1.51 versus 1.13, p = 0.02), lower fractional area change (17.65 versus 33.99, p < 0.01), lower global longitudinal strain (GLS) (-8.63 versus - 17.99, p < 0.01), lower global longitudinal strain rate (GLSR) (- 0.51 versus - 0.93, p < 0.01), lower global circumferential strain (GCS) (-6.68 versus -18.25, p < 0.01), and lower (GCSR) global circumferential strain rate (-0.45 versus -1.01, p < 0.01). ROC analysis demonstrated predictive value for GLS - 7.6 (71% sensitive, 97% specific, AUC 81%), GLSR -0.58 (71% sensitive, 88% specific, AUC 82%), GCS - 10.0 (86% sensitive, 91% specific, AUC 82%), and GCSR -0.85 (100% sensitive, 71% specific, AUC 90%). GLS and GCS can help predict transplant-free survival in patients with hypoplastic left heart syndrome having undergone Fontan palliation. Higher strain values (closer to zero) may be a helpful tool in determining when transplant evaluation is warranted in these patients.

Comparison of Quantitative Versus Qualitative Assessment of Single Right Ventricular Function for the Prediction of Midterm Outcomes.

Multiple studies have shown that quantitative evaluation of right ventricular (RV) systolic function in children with hypoplastic left heart syndrome (HLHS) is associated with outcomes. However, the most widely used method is qualitative, or subjective echocardiographic evaluation. Tricuspid annular displacement (TMAD) is a quantitative method and has been shown to be associated with transplant/death in a cohort of pediatric patients with HLHS. In this study, the same echocardiograms used in the quantitative TMAD study were qualitatively evaluated to see if the assessment correlated with midterm outcomes. We hypothesized that TMAD measures would outperform qualitative measurements. A previously published retrospective study of patients with systemic right ventricle demonstrated that TMAD measurements of function prior to the Glenn procedure were associated with midterm mortality/transplant (mean TMAD 12.4% for survivors and 10.0% for non-survivors/transplant, p = 0.03). Echocardiographic images used in that study were re-evaluated using qualitative assessment of function. A score between severely depressed (0) and supra-normal (7) was assigned independently by three cardiologists. A chart review included short-term clinical outcome measures in addition to midterm mortality/transplant. Spearman correlations and logistic regression were used to estimate the associations between function scores and clinical outcomes. Function scores were measured with acceptable inter- and intra-rater reliabilities in 47 patients who had echocardiograms used in the prior analysis before their bidirectional Glenn. The mean functional score was 5.3 ± 1.32 in survivors and 5.0 ± 1.02 in non-survivors/transplant. Subjective echocardiographic scores of function were not predictive of mortality (p = 0.23). Scores were correlated with length of cardiac intensive care unit stay (Spearman's rho = - 0.31, p = 0.04), but not post-Glenn total length of hospital stay (p = 0.4). A sub-analysis was performed for each individual operator. Only one of three operators produced scores with a significant association with mortality/transplant (p = 0.01, p = 0.25, p = 0.22, respectively). Averaged subjective measurement of function by three pediatric cardiologists in children with single right ventricle prior to the Glenn procedure was not associated with midterm outcomes. Previous work based on the same echocardiograms showed that quantitative analysis was associated with midterm outcomes. This suggests quantitative analysis may be more useful in prognostication. Future studies could confirm these results and identify which quantitative methods are most helpful.

Added Value of Live 3-Dimensional Transesophageal Echocardiography During Procedures in Fontan Patients.

3D transesophageal echocardiography (3D-TEE) has proven useful and accurate during some operating room (OR), interventional cardiac catheterization (Cath), and electrophysiologic (EP) procedures. The use of 3D-TEE during similar procedures in patients who have undergone Fontan surgery and its additional value have not been previously reported. To determine if live 3D-TEE during procedures post Fontan has added value, 3D-TEEs in 58 post-Fontan patients over a 5-year study period were reviewed. Additional information provided by 3D-TEE (over 2D-TEE) was classified into the following: A: new information which changed/refined the plan and 0: no new important information. Pre- and post-bypass OR 3D-TEEs were counted as one study. A total of 67 3D-TEEs (41 Cath, 13 OR, and 13 EP procedures) were performed. Median age was 14 years (6-39 years). Median weight was 47 kg (21-109 kg). In Cath procedures, only 2/41 (5%) were graded A (R to L atrial level shunt [Fontan leak], n = 1; thrombus in pulmonary artery stump, n = 1). In the OR, 6/13 (46%) were graded A (atrioventricular valvuloplasty, n = 1; neo-aortic valvuloplasty, n = 1; relief of systemic and pulmonary venous outflow obstruction, n = 2 and n = 2; respectively). In EP procedures, 4/13 (31%) were graded A (thrombus, n = 3; mapping for lead placement to assist in multisite pacing for dyssynchrony, n = 1). 3D-TEE of Fontan improved visualization and frequently added value in the OR/EP lab and may be helpful in select catheterization cases. Future studies with a larger sample could build on this data to identify when 3D-TEE will be most useful.

Comparison of Semi-Automated Versus Manual Quantitative Right Ventricular Assessment in Hypoplastic Left Heart Syndrome.

Quantitative echocardiographic evaluation is important for systemic right ventricles, but its asymmetric shape makes this challenging and time consuming when performed manually. Semi-automated software could make these quantitative measures easier to accomplish in the clinical setting. We hypothesized that semi-automated software would approximate manual measures of right ventricular size and function. Children with hypoplastic left heart who had echocardiograms were prospectively identified. These measurements were performed using manual and semi-automated techniques: end-diastolic and end-systolic area, fractional area change (FAC), dimensions (longitudinal, basal and mid-cavitary diameters), and tricuspid annular plane systolic excursion (TAPSE). Agreement between measures was evaluated. Sixty-three echocardiograms were analyzed. Intra- and inter-observer reliability was acceptable and similar between methods except that inter-observer reliability for the manual method was superior for TAPSE. Correlation between methods was high (r > 0.9, p < 0.001) for most of the measures. Correlation for FAC was r = 0.79, and for TAPSE the correlation was r = 0.61 (both p < 0.001). The percent relative difference between manual and semi-automated methods was less than 6% for most measures. End-systolic area and FAC had a relative difference of 10% and 11% respectively. The only measure with substantial bias between the manual and semi-automated methods was TAPSE which had a relative difference of 52%. EchoInsight® semi-automated software provides similar measures of right ventricular dimensions and FAC in patients with hypoplastic left heart compared to manual measures. Measures of TAPSE do not correlate well between manual and semi-automated methods. Further research is warranted on the use of semi-automated analyses in this patient population.

Right Ventricular Mechanical Dyssynchrony in Hypoplastic Left Heart Syndrome: Correlation with Systolic Function and QRS Duration.

The single right ventricle (RV) in hypoplastic left heart syndrome (HLHS) often develops systolic dysfunction with time and this affects prognosis. Mechanical dyssynchrony has been reported in HLHS but has not consistently correlated with systolic function or electrical dyssynchrony. The aims of this study were to assess the relationship between RV mechanical dyssynchrony, RV systolic function, and QRS duration on surface electrocardiography. We hypothesized that surface QRS duration would not be an adequate indicator of mechanical dyssynchrony compared with dyssynchrony parameters. Retrospective analysis of echocardiograms of patients with HLHS divided into preserved vs reduced RV function. We measured two RV function parameters: (1) fractional area change (FAC) and (2) global longitudinal strain (RVGLS). We measured two dyssynchrony parameters: (1) the standard deviation of the time to peak strain for 9 segments (tPS-9) and (2) time difference between the earliest and latest time to peak strain (RV dyssynchrony index or RVDI) both corrected for R-R interval. We also measured the QRS duration from surface EKG. Mechanical dyssynchrony parameters were compared to both RV systolic function and to QRS duration. 41 patients with HLHS were identified: 21 had preserved function and 20 had reduced function defined by a FAC < 35%. The reduced function group had a significantly lower mean FAC and RVGLS. RVDI was higher in the dysfunction group and had a modest correlation with FAC (r = 0.48) and RVGLS (r = 0.57). tPS-9 was longer in the dysfunction group and had a modest correlation with FAC (r = 0.45) and RVGLS (r = 0.57). QRS duration was longer in the dysfunction group and had a modest correlation with FAC (r = 0.56) and RVGLS (r = 0.56). The weakest correlations were between QRS duration and tPS-9 (r = 0.32) and QRS duration and RVDI (r = 0.10). RV dysfunction measured by FAC was associated with mechanical dyssynchrony measured by increased RVDI and tPS-9. QRS duration was longer in the group with dysfunction but did not correlate with directly measured mechanical dyssynchrony. This may have potential implications for cardiac resynchronization therapy in univentricular patients as assessed by mechanical dyssynchrony parameters rather than QRS duration alone.

Saline Contrast Transesophageal Echocardiography in Fontan Patients: Assessment of the Presence, Type, and Size of Right to Left Shunts.

Right to left (R-L) shunts resulting in cyanosis or systemic embolization occur after the Fontan procedure. The primary modality of diagnosing these is angiography. Successful delineation of these shunts in Fontan patients using selective saline contrast transesophageal echocardiography (SCTEE) may allow for reduced radiation and contrast exposure. We hypothesized that SCTEE could accurately determine the presence, type, and semiquantitative shunt size of R-L shunts in Fontan patients. SCTEE was performed in Fontan patients undergoing angiography for clinical indications. Injections were performed in six sites: mid-Fontan, right and left pulmonary arteries, superior and inferior vena cavae, and innominate vein. R-L shunt size was subjectively graded as 0 = absent, 1 = small, and 2 = medium or large based on echo contrast density in the left atrium. SCTEE was compared to angiography. 33 patients with Fontan were studied with median age 15 years, median weight 50.1 kg, and median O2 saturation of 90% in the R-L shunt group and 95% in the no R-L shunt group. R-L shunt types included intracardiac shunts (ICS), veno-venous collaterals (VVCs), arteriovenous malformations (AVMs), and their combinations. SCTEE versus angiography results were the same for the presence, type, and size of R-L shunts in 79% (26/33). SCTEE identified shunts in 88% (29/33). Angiography identified shunts in 85% (28/33). Neither method missed any medium or large R-L shunts. SCTEE and angiography had similar accuracy. SCTEE accurately detected the presence, type, and size of R-L shunts in most Fontan patients in this study. This can be used to guide targeted angiography, reducing radiation exposure and contrast load.

Right Ventricular Systolic Function Parameters in Hypoplastic Left Heart Syndrome.

Assessment of the systolic function of the right ventricle (RV) in patients with hypoplastic left heart syndrome (HLHS) is important. The asymmetric shape and heavy trabeculations make accurate assessment of RV systolic function challenging. Novel measures of RV function could be helpful in distinguishing reduced versus preserved function in HLHS and may also be worse in HLHS with preserved function compared to normal controls. These novel methods offer promise, but research and clinical applicability is hindered as no cut-off values for normal function have been established. We performed a retrospective comparison of functional assessments from echocardiograms of HLHS patients with preserved and reduced RV function along with a control group of normal patients. Measures of function included fractional area change (FAC), tissue motion annular displacement of the tricuspid (TMAD-TV) and pulmonary valves (TMAD-PV), myocardial performance index (MPI), tricuspid tissue Doppler S' velocity, and RV global longitudinal strain (RVGLS). Comparisons were made between three groups: normal patients, HLHS with preserved function, and HLHS with reduced function defined as FAC < 35%. FAC was chosen as the reference as it is a historical standard. 41 HLHS patients were studied. Of these patients, 20 had HLHS with reduced function, and 21 had preserved function. They were compared with 27 age-matched, normal, controls. Comparison between HLHS and normal controls: in HLHS with preserved RV systolic function, compared to normal controls, tissue Doppler S', MPI, and TMAD-TV were all abnormal (all p < 0.05). RVGLS was not statistically different (20.5 ± 3.6% for normal vs. 17.9 ± 2.6% for HLHS with preserved function). TMAD-PV was similar between groups (16.1 ± 4.6% vs. 16.7 ± 5.1%). All measures were significantly worse (all p < 0.05) in the HLHS with reduced function group compared to normal controls. Comparison between HLHS preserved vs reduced function: in HLHS with reduced function defined by FAC < 35%, all measures were significantly worse compared to HLHS with preserved function (all p < 0.05). The cut-off values that correspond to a FAC of > 35% were 14.5% for TMAD-TV and 16% for RVGLS. All measures except RVGLS and TMAD-PV estimated worse function than controls even for HLHS with preserved function. Each of the functional measures was able to identify preserved vs reduced function in HLHS with FAC as the reference standard. Cut-off values between preserved and reduced function in HLHS were estimated for TMAD-TV and RVGLS based on a relatively small cohort. These cut-off values will aid in the research design of future studies.

Right Ventricular Longitudinal Shortening Before and After Stage I Surgical Palliation Correlates with Outcomes.

Quantification of right ventricular function is difficult, but important, in patients with single ventricles. Tissue motion annular displacement (TMAD) is an echocardiographic tool that measures displacement of the tricuspid valve relative to the apex. We evaluated TMAD, lateral annular displacement (LAD), and fractional area change (FAC) for correlation with outcomes. We measured TMAD, LAD, FAC, and other variables that may affect prognosis in patients with single right ventricle physiology pre- and post-Stage I palliation and correlated them with outcomes up to the Glenn procedure. Intra- and inter-observer variability for TMAD measurements were 2.7% (1.2-3.5%) and 6.1% (3.3-8.1%), respectively. Sixty-six subjects met the inclusion criteria. Pre-Stage I TMAD was 13.7% (SD 3.9%). TMAD had a linear relationship with FAC (r2 = 0.76). There was a correlation between TMAD and hospital stay (p = 0.044) and ECMO/arrest (p = 0.024). LAD correlated with ECMO/arrest (p = 0.045) and mortality/transplant (p = 0.049). FAC correlated with in-hospital mortality (p = 0.028). Post-Stage I TMAD was 11.8% (SD 3.7%). TMAD, LAD, and FAC all correlated with in-hospital mortality and mortality/transplant. In multivariate models, TMAD was independently predictive of weight for age Z score pre-Glenn. TMAD, FAC, and LAD correlate with clinically significant outcomes after the first-stage palliation. TMAD correlated with more outcomes than FAC and was the only measure that was independently predictive of any outcome. TMAD is a reproducible measure of RV function in this population. TMAD has prognostic value before and after first-stage palliation and may outperform more traditional measures.

Gemella bergeri infective endocarditis: a case report and brief review of literature.

Gemella is a genus of Gram-positive bacteria found in the digestive tract of humans. They rarely cause systemic illness but have been recently implicated in several serious infections. We report infective endocarditis caused by Gemella bergeri in a 23-year-old with a bicuspid aortic valve status post-intervention in infancy.

Hemodynamic impact of coronary stenosis using computed tomography: comparison between noninvasive fractional flow reserve and 3D fusion of coronary angiography with stress myocardial perfusion.

Vasodilator-stress CT perfusion imaging in addition to CT coronary angiography (CTCA) may provide a single-test alternative to nuclear stress testing, commonly used to assess hemodynamic significance of stenosis. Another alternative is fractional flow reserve (FFR) calculated from cardiac CT images. We studied the concordance between these two approaches and their relationship to outcomes. We prospectively studied 150 patients with chest pain, who underwent CTCA and regadenoson CT. CTCA images were interpreted for presence and severity of stenosis. Fused 3D displays of subendocardial X-ray attenuation with coronary arteries were created to detect stress perfusion defects (SPD) in each coronary territory. In patients with stenosis > 25%, CT-FFR was quantified. Significant stenosis was determined by: (1) combination of stenosis > 50% with an SPD, (2) CT-FFR ≤ 0.80. Patients were followed-up for 36 ± 25 months for death, myocardial infarction or revascularization. After excluding patients with normal arteries and technical/quality issues, in final analysis of 76 patients, CTCA depicted stenosis > 70% in 13/224 arteries, 50-70% in 24, and < 50% in 187. CT-FFR ≤ 0.80 was found in 41/224 arteries, and combination of SPD with > 50% stenosis in 31/224 arteries. Inter-technique agreement was 89%. Despite high incidence of abnormal CT-FFR (30/76 patients), only 7 patients experienced adverse outcomes; 6/7 also had SPDs. Only 1/9 patients with CT-FFR ≤ 0.80 but normal perfusion had an event. Fusion of CTCA and stress perfusion can help determine the hemodynamic impact of stenosis in one test, in good agreement with CT-FFR. Adding stress CT perfusion analysis may help risk-stratify patients with abnormal CT-FFR.

A novel approach to the diagnosis and treatment of hemoptysis in infants: A case series.

INTRODUCTION: Hemoptysis in children is an uncommon presenting symptom but can be life-threatening if massive. Cardiac catheterization and coil embolization of aorto-pulmonary collateral vessels (APCs) is uncommon in pediatric hemoptysis patients without congenital heart disease. METHODS: We present a series of seven infants (≤12 months of age) with hemoptysis, all of whom underwent cardiac catheterization to look for and intervene upon APCs, if found. Only those patients who underwent both bronchoscopy as well as cardiac catheterization from January 1995 to January 2015 were included in this retrospective review. RESULTS: Seven patients met inclusion criteria, and three had a history of recurrent hemoptysis. The mean age was 3 months. Four had evidence of bleeding on bronchoscopy. All seven had respiratory distress which necessitated ICU admission; five required mechanical ventilation. Cardiac catheterization showed significant APCs (>2 mm) in six of the seven studied patients, all of which were coil embolized. One patient had no significant APCs and therefore, no embolization. All patients had complete resolution with no recurrences during the 10-20-year outpatient follow-up period. Chest CT scans were not helpful in delineating the site or etiology of bleeding in any patient. CONCLUSIONS: APCs should be considered as a differential diagnosis for pulmonary hemorrhage in infants after more common causes have been ruled out.

Tissue Motion Annular Displacement Predicts Mortality/Transplant After the Bidirectional Glenn.

BACKGROUND: Quantifying right ventricular function in patients with a systemic right ventricle (RV) is difficult but important for prognosis. Tissue motion annular displacement tracks displacement of the tricuspid annulus toward the apex. We evaluated this measure alongside fractional area change (FAC) on patients with single, RV prior to the bidirectional Glenn procedure. We tested both measures for correlation with outcomes. METHODS: Retrospective measurement of tissue motion annular displacement and FAC was performed on echocardiographic clips obtained prior to the bidirectional Glenn. A chart review included postoperative outcomes and midterm mortality/transplant. Bivariate correlations and Cox proportional hazards models were used for analyses. RESULTS: Fifty-one patients with dominant RV underwent the bidirectional Glenn procedure and all had image quality that allowed analysis. The age ranged from 3 to 11 months (median 4 months). Neither tissue motion annular displacement nor FAC correlated with short-term postoperative outcomes. Tissue motion annular displacement was independently predictive of mortality/transplant ( P = .03) in the Cox hazard model. The mean for survivors was 12.4% and for nonsurvivors/transplants was 10.0%. Tissue motion annular displacement intra-observer variability was 2.8% (1.2%-3.5%). Interobserver mean variability was 6.1% (3.3%-8.1%). Fractional area change was not predictive of mortality/transplant. CONCLUSION: Tissue motion annular displacement is an independent predictor of midterm mortality/transplant after the bidirectional Glenn procedure in patients with single, RV, in this study. It may outperform FAC in this regard and has good reproducibility. Tissue motion annular displacement may be a useful measure in identifying high-risk children in this population.

Fusion of Three-Dimensional Echocardiographic Regional Myocardial Strain with Cardiac Computed Tomography for Noninvasive Evaluation of the Hemodynamic Impact of Coronary Stenosis in Patients with Chest Pain.

BACKGROUND: Combined evaluation of coronary stenosis and the extent of ischemia is essential in patients with chest pain. Intermediate-grade stenosis on computed tomographic coronary angiography (CTCA) frequently triggers downstream nuclear stress testing. Alternative approaches without stress and/or radiation may have important implications. Myocardial strain measured from echocardiographic images can be used to detect subclinical dysfunction. The authors recently tested the feasibility of fusion of three-dimensional (3D) echocardiography-derived regional resting longitudinal strain with coronary arteries from CTCA to determine the hemodynamic significance of stenosis. The aim of the present study was to validate this approach against accepted reference techniques. METHODS: Seventy-eight patients with chest pain referred for CTCA who also underwent 3D echocardiography and regadenoson stress computed tomography were prospectively studied. Left ventricular longitudinal strain data (TomTec) were used to generate fused 3D displays and detect resting strain abnormalities (RSAs) in each coronary territory. Computed tomographic coronary angiographic images were interpreted for the presence and severity of stenosis. Fused 3D displays of subendocardial x-ray attenuation were created to detect stress perfusion defects (SPDs). In patients with stenosis >25% in at least one artery, fractional flow reserve was quantified (HeartFlow). RSA as a marker of significant stenosis was validated against two different combined references: stenosis >50% on CTCA and SPDs seen in the same territory (reference standard A) and fractional flow reserve < 0.80 and SPDs in the same territory (reference standard B). RESULTS: Of the 99 arteries with no stenosis >50% and no SPDs, considered as normal, 19 (19%) had RSAs. Conversely, with stenosis >50% and SPDs, RSAs were considerably more frequent (17 of 24 [71%]). The sensitivity, specificity, and accuracy of RSA were 0.71, 0.81, and 0.79, respectively, against reference standard A and 0.83, 0.81, and 0.82 against reference standard B. CONCLUSIONS: Fusion of CTCA and 3D echocardiography-derived resting myocardial strain provides combined displays, which may be useful in determination of the hemodynamic or functional impact of coronary abnormalities, without additional ionizing radiation or stress testing.

Three-dimensional analysis of regional right ventricular shape and function in repaired tetralogy of Fallot using cardiovascular magnetic resonance.

BACKGROUND: Patients with surgically repaired tetralogy of Fallot (rTOF) often have residual pulmonic valve regurgitation, leading to abnormal remodeling and dysfunction of the right ventricle often requiring pulmonic valve replacement. We tested the hypothesis that 3D analysis of right ventricular (RV) shape and function may reveal differences in regional adaptive remodeling that occurs in patients with rTOF, depending on whether a transannular patch (TAP) was utilized. METHODS: Forty patients with rTOF who underwent cardiac magnetic resonance imaging (1.5 T), including 20 with and 20 without TAP, and 10 normal controls were studied. Images were analyzed to measure RV endocardial curvature and global and regional volume and function. RESULTS: RV ejection fraction (EF) was 42 ±â€¯11% in TAP and 38 ±â€¯9% in no-TAP (p = 0.19), both lower than 54 ±â€¯3% in controls (p < 0.01). Left ventricular (LV) EF was 54 ±â€¯9% in TAP, 54 ±â€¯8% in no-TAP (p = 0.87) and 61 ±â€¯16% in controls (both p < 0.01). Indexed LV end-diastolic volumes were higher in no-TAP than in TAP subgroup (p = 0.02). With TAP, mid RV septum showed lower curvature during diastole (less convex), than the mid and apical free walls and free wall adjacent to the RV outflow tract (RVOT; more convex). There were no differences in curvature during systole between rTOF subgroups but mid and RVOT free walls showed higher curvature versus controls. CONCLUSIONS: This is the first study to comprehensively describe the influence of TAP on changes in regional RV shape in patients with rTOF. Understanding these differences may help guide therapeutic options for residual pulmonary valve regurgitation in rTOF patients.

Acquired Cor Triatriatum Sinister After the Fontan Operation and Successful Resection in a Child.

Cor triatriatum sinister is a rare congenital lesion encountered in children. It consists of a fibromuscular membrane that separates the left atrium into two chambers resulting in a triatrial heart and often occurs with other structural cardiac anomalies. The acquired form is uncommon and has only been reported after orthotopic heart transplantation or as a complication of infective endocarditis in adults. These cases were mostly because of hypertrophied atrial tissue or suture lines and torsion of the atrium. We describe the first case of acquired cor triatriatum late after the Fontan procedure with successful surgical resection in a child.