Hybrid strategies for high-risk non-hypoplastic left heart syndrome patients.

Single-stage repair of complex cardiac abnormalities in high-risk neonates presents formidable challenges. The majority of hybrid strategies involving bilateral pulmonary artery banding (bPAB) with or without patent ductus arteriosus (PDA) stenting is described in the setting of hypoplastic left heart syndrome. We present a series of cases describing two-stage repair with initial palliative hybrid procedures involving bPAB with or without PDA stenting. This allows weight gain and stabilization of circulation before complete repair, provides good results, and may overcome risk factors associated with single-stage repair in neonates.

The angulation of the septal structures impacts ventricular imbalance in atrioventricular septal defects with a common atrioventricular junction.

OBJECTIVE: Multiplanar re-formatting of full-volume three-dimensional echocardiography data sets offers new insights into the morphology of atrioventricular septal defects. We hypothesised that distortion of the alignment between the atrial and ventricular septums results in imbalanced venous return to the ventricles, with consequent proportional ventricular hypoplasia. METHODS: A single observer evaluated 31 patients, with a mean age of 52.09 months, standard deviation of 55, and with a range from 2 to 264 months, with atrioventricular septal defects, of whom 17 were boys. Ventricular imbalance, observed in nine patients, was determined by two-dimensional assessment, and confirmed at surgical inspection in selected cases when a univentricular strategy was undertaken. Offline analysis using multiplanar re-formatting was performed. A line was drawn though the length of the ventricular septum and a second line along the plane of the atrial septum, taking the angle between these two lines as the atrioventricular septal angle. We compared the angle between 22 patients with adequately sized ventricles, and those with ventricular imbalance undergoing univentricular repair. RESULTS: In the 22 patients undergoing biventricular repair, the septal angle was 0 in 14 patients; the other eight patients having angles ranging from 1 to 36, with a mean angle of 7.4°, and standard deviation of 11.1°.The mean angle in the nine patients with ventricle imbalance was 28.6°, with a standard deviation of 3.04°, and with a range from 26 to 35°. Of those undergoing univentricular repair, two patients died, with angles of 26 and 30°, respectively. CONCLUSIONS: The atrioventricular septal angle derived via multiplanar formatting gives important information regarding the degree of ventricular hypoplasia and imbalance. When this angle is above 25°, patients are likely to have ventricular imbalance requiring univentricular repair.

Foetal supraventricular tachycardia with hydrops fetalis: a role for direct intraperitoneal amiodarone.

INTRODUCTION: Persistent foetal tachyarrythmias complicated by hydrops fetalis carry a poor prognosis, with foetal death reported in excess of a quarter despite treatment. We present our experience with direct intraperitoneal amiodarone administration in eight hydropic foetuses with resistant supraventricular tachycardia. METHODS: Amiodarone was injected slowly into foetal peritoneal cavity under ultrasound guidance. All mothers were loaded with oral amiodarone before the procedure and maintained on it. The procedure was repeated guided by foetal rhythm. RESULT: All eight cases had severe hydrops with a median foetal heart rate of 255 bpm (range 240-300 bpm), and the median gestational age was 27+1 weeks (range 21-33+3 weeks) at presentation. In six cases, the average time for supraventricular tachycardia to revert to sinus rhythm from the first procedure was 11.5 days. In one case, intravascular injection of amiodarone into the umbilical vein was performed before intraperitoneal injection, which resulted in conversion to sinus rhythm sustained until delivery. In the last case, supraventricular tachycardia and severe hydrops persisted and the baby was delivered 5 days later at 34 weeks' gestation. Hydrops resolved in five foetuses with a mean resolution time of 28.4 days. The mean gestational age at delivery was 34+5 days and seven of eight cases survived beyond the neonatal period with good postnatal outcomes. CONCLUSION: Intraperitoneal administration of amiodarone is a relatively simple and effective strategy in refractory supraventricular tachycardia complicated by severe hydrops. The intraperitoneal route assures delivery of the drug to the severely hydropic foetus and enables a bolus dose to be delivered for sustained absorption.

Midterm evaluation of biological prosthetic valves in the pulmonary position of grown-up patients.

OBJECTIVES: To examine the midterm clinical outcome of pulmonary valve replacement (PVR) with prosthetic valves. METHODS: We reviewed 37 consecutive patients who underwent PVR with biological prosthetic valves between September 1999 and June 2010. The median age was 22.6 years (range: 6 to 70 years; three children). The primary diagnosis was Tetralogy of Fallot in 20 patients (54%). Valve pathology was regurgitation in 27 patients (72.9%). Cardiac surgery had been previously performed in 35 patients (94.5%). The median size of the prosthesis was 25 mm (range: 21 to 31 mm). The median follow-up was 42 months (range: 1.2 to 129 months). RESULTS: There were no early valve-related deaths. Hospital mortality was 2.7% (n = 1) and no patient required early rereplacement of prosthesis. Two patients required permanent pacemaker insertion. During follow-up, there was no late death, reoperation for structural valve degeneration, or valve thrombosis. Only one patient required repeated operation for endocarditis at 37 months follow-up. The actuarial survival at 5 years was 95.1 ± 3.8%. Overall freedom from reoperation after PVR at 5 years was 93.0 ± 8.6%. At last follow-up, 34 patients (91.8%) were NYHA class I versus 20 patients (54%) preoperatively (p < 0.05). In the 35 surviving patients who did not undergo redo-PVR, there was no-mild regurgitation and the peak PV gradient was 20.4 ± 10.2 mm Hg (16.2 ± 9.3 mm Hg preoperatively). Thirty-two patients (91.4%) had good right ventricular function compared with 26 patients (74.2%) preoperatively. CONCLUSIONS: PVR with biological prosthetic valves can be performed with good midterm survival, functional status, and haemodynamics.

Inhaled Nitric Oxide in Acute Severe Pulmonary Hypertension and Severe Acute Respiratory Distress Syndrome Secondary to COVID-19 Pneumonia: A Case Report.

BACKGROUND Inhaled nitric oxide (iNO) is used as a treatment for pulmonary arterial hypertension (PAH). Severe hypoxia with hypoxic vasoconstriction caused by severe acute respiratory distress syndrome (ARDS) can induce pulmonary hypertension with hemodynamic implications, mainly secondary to right ventricle (RV) systolic function impairment. We report the case of the use of iNO in a critically ill patient with bilateral SARS-CoV-2 pneumonia and severe ARDS and hypoxemia leading to acute severe PAH, causing a ventilation/perfusion mismatch, RV pressure overload, and RV systolic dysfunction. CASE REPORT A 36-year-old woman was admitted to the Intensive Care Unit with a severe ARDS associated with SARS-CoV-2 pneumonia requiring invasive mechanical ventilation. Severe hypoxia and hypoxic vasoconstriction developed, leading to an acute increase in pulmonary vascular resistance, severe to moderate tricuspid regurgitation, RV pressure overload, RV systolic function impairment, and RV dilatation. Following 24 h of treatment with iNO at 15 ppm, significant oxygenation and hemodynamic improvement were noted, allowing vasopressors to be stopped. After 24 h of iNO treatment, echocardiography showed very mild tricuspid regurgitation, a non-dilated RV, no impairment of transverse free wall contractility, and no paradoxical septal motion. iNO was maintained for 7 days. The dose of iNO was progressively decreased with no adverse effects and maintaining an improvement of oxygenation and hemodynamic status, allowing respiratory weaning. CONCLUSIONS Sustained acute hypoxia in ARDS secondary to SARS-CoV-2 pneumonia can lead to PAH, causing a ventilation/perfusion mismatch and RV systolic impairment. iNO can be considered in patients with significant PAH causing hypoxemia and RV dysfunction.

Determinants of outcome in fetal pulmonary valve stenosis or atresia with intact ventricular septum.

Pulmonary valve stenosis or atresia with intact ventricular septum represents a spectrum of severity. This study aimed to identify ultrasound markers of biventricular versus non-biventricular outcome. The fetal echocardiograms of 41 fetuses diagnosed with pulmonary stenosis or atresia and right ventricular (RV)/left ventricular (LV) length ratios >0.4 from 17 to 31 weeks of gestation were reviewed. Of 27 live-born patients with intention to treat, 8 had non-biventricular outcomes and 19 had biventricular circulation. At the time of diagnosis, poor RV function, flow reversal in the arterial duct, the degree of tricuspid valve (TV) regurgitation, and inferior vena cava Doppler flow pattern did not differ between the 2 outcome groups. However, RV sinusoids, the RV/LV length ratio, the TV/mitral valve ratio, and TV inflow duration were significantly different. Cut-off values derived from receiver-operating characteristic curves yielding the best sensitivity and specificity for a non-biventricular outcome were TV/mitral valve ratio <0.7, RV/LV length ratio <0.6, TV inflow duration <31.5% of cardiac cycle length, and the presence of RV sinusoids. If 3 of these 4 criteria were fulfilled, this predicted a non-biventricular outcome with sensitivity of 100% and specificity of 75%. In conclusion, in fetuses < or =31 weeks of gestation with pulmonary stenosis or atresia and intact ventricular septum, progression to a non-biventricular outcome can be predicted by a 4-criterion scoring system. The criteria may be useful in selecting fetuses for prenatal catheter intervention to prevent progressive RV hypoplasia.

Differential regurgitation in branch pulmonary arteries after repair of tetralogy of Fallot: a phase-contrast cine magnetic resonance study.

BACKGROUND: The importance of pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) is increasingly recognized, but little is known regarding its underlying mechanisms. This phase-contrast cine magnetic resonance (PC MR) study was performed to evaluate the relative contribution of each lung to total regurgitant volume. METHODS AND RESULTS: Twenty-two patients with significant PR underwent a PC MR 3 to 16 years after repair of TOF. Regurgitant fraction of the main pulmonary artery was 39+/-10%. Regurgitant fraction of the left pulmonary artery (LPA; 46+/-18%) was greater than that of the right pulmonary artery (34+/-16%; P=0.002). The average contribution of the LPA to the total regurgitant flow volume was 54+/-19%, whereas its average contribution to the total forward flow volume was 44+/-13% (P=0.002). In 4 patients, regurgitant flow in the LPA accounted for 75% to 100% of the total regurgitant flow. There was a linear relationship between regurgitant fraction and fraction of the regurgitant flow duration in the main pulmonary artery (P<0.001) and right pulmonary artery (P=0.001) but not in the LPA (P=0.129). CONCLUSIONS: PR after repair of TOF is commonly associated with differential regurgitation in the branch pulmonary arteries, which is usually greater in the LPA. Although the cause of this disparity requires further investigation, those patients with a significant unilateral contribution to total PR may be amenable to localized techniques to reduce regurgitation.

Three-dimensional echocardiography improves the understanding of left atrioventricular valve morphology and function in atrioventricular septal defects undergoing patch augmentation.

OBJECTIVES: We sought to address the role of 3-dimensional echocardiography in the evaluation of the left atrioventricular valve in children with an atrioventricular septal defect who underwent patch augmentation of their valve for either regurgitation or left ventricular outflow tract obstruction. METHODS: Five children whose ages ranged between 4.5 and 9.2 years and who underwent patch augmentation of their left atrioventricular valve had a preoperative and postoperative transesophageal echocardiogram with 3-dimensional reconstruction to evaluate the left atrioventricular valve. The indication for operation was left atrioventricular valve regurgitation in 3 patients and left ventricular outflow tract obstruction in 2 patients. Three were rerepairs, and 2 were primary repairs. Both 3-dimensional morphology and color Doppler data were obtained. Two- and 3-dimensional findings were correlated with surgical observations through the use of direct inspection and video images obtained with a head-mounted super-VHS camera. RESULTS: In each case there was precise correlation between the 3-dimensional and surgical findings as to the cause of leaflet failure in those with regurgitation. The site that would require leaflet augmentation could be determined by means of 3-dimensional echocardiography. Three-dimensional echocardiography provided more specific detail as to the morphology and function of the left atrioventricular valve than did its 2-dimensional counterpart. CONCLUSIONS: Three-dimensional echocardiography provides detailed information about the status of the left atrioventricular valve in the atrioventricular septal defect and can aid in the planning of either primary or secondary repair.

Obstructed hemianomalous pulmonary venous drainage: is intervention necessary?

Obstructed infradiaphragmatic hemianomalous pulmonary venous drainage is associated with high mortality and morbidity, and treatment is best tailored to the individual patient. Resolution of an obstructed scimitar vein through collateralization has rarely been reported. We report two such cases and review the literature.

Intrapericardial teratoma in neonates: a surgical emergency.

Primary cardiac tumors are very rare, with a reported incidence of 0.15% to 0.2% in autopsy series. They can be life threatening because of myocardial compression and ventricular dysfunction. Once diagnosed during pregnancy, the clinical condition of the baby is monitored because of the risk of rupture of the tumor capsule. The authors report a rare case of a neonate who presented with respiratory and cardiac compromise due to cardiac tamponade necessitating emergency exploration of the pericardium and excision of tumor. A well-encapsulated tumor measuring around 5 cm and bigger than the heart was completely excised. This was diagnosed to be an immature teratoma. Follow-up echocardiogram was normal, and on serial monitoring, alpha-fetoprotein was within normal limits. The baby was discharged home with no complications. Intrapericardial teratoma in neonates is a surgical emergency if presented with significant pericardial effusion. It can be a challenge if diagnosed in utero with rupture before the viability of pregnancy. A multidisciplinary team approach is necessary to manage such situations. Complete excision is necessary because of its association with tissues of malignant potential.

Primary biventricular repair of atrioventricular septal defects: an analysis of reoperations.

BACKGROUND: The purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair. METHODS: Between April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle. RESULTS: There was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% +/- 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome (p = 0.01) and the presence of right ventricular dominance (p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients. CONCLUSIONS: Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality.

Long-term follow-up after primary complete repair of common arterial trunk with homograft: a 40-year experience.

BACKGROUND: We sought to determine the long-term performance of homograft and truncal valve after complete repair of common arterial trunk. METHODS: From January 1964 to June 2008, 32 patients (median age, 14 days; range, 5 days to 2.5 years) underwent primary homograft repair of common arterial trunk. Twenty-four (75%) were neonates. The homograft used in the right ventricular outflow tract was aortic in 24 patients and pulmonary in 8 patients (mean diameter, 15.8 +/- 3.5 mm; median diameter, 16 mm [range, 8-24 mm]). The median follow-up was 24.5 years (range, 5.6 months to 43.5 years). RESULTS: There were 3 hospital deaths and 1 late death. The actuarial survival at 30 years was 83.1% +/- 6.6%. Of the 28 survivors, 25 reoperations were performed in 19 (76%) patients. The mean and median times to homograft reoperation were 11.5 +/- 7.4 and 12.1 years (range, 1.0-26.1 years), respectively. Overall freedom from homograft reoperation after 10, 20, and 30 years was 68.4% +/- 8.7%, 37.4% +/- 9.5%, and 26.7% +/- 9.3%, respectively. Twelve patients retained the original homografts at a median follow-up of 16.4 years (range, 0-30.2 years). Six underwent a truncal valve replacement with a mechanical prosthesis at a median of 10.5 years (range, 3.4-22 years) after truncus repair. Freedom from truncal valve replacement at 10 and 30 years was 93.1% +/- 4.7% and 81.8% +/- 8.9%, respectively. In the 22 surviving patients who did not undergo truncal valve replacement, the peak truncal valve gradient was 8.9 +/- 8.3 mm Hg at a median follow-up of 24.5 years (range, 5.6 months to 32.9 years). At the last follow-up, 27 (96.4%) patients had good left ventricular function, and 24 patients (85.7%) were New York Heart Association class I. CONCLUSIONS: Oversizing the homograft at the time of the initial repair can lead to a homograft lasting more than 12 years. During long-term follow-up, 20% of patients require truncal valve replacement.

Impact of multiplanar review of three-dimensional echocardiographic data on management of congenital heart disease.

BACKGROUND: In patients with congenital cardiac malformations, accurate diagnosis is critical in diagnosis and management. The multiplanar review mode (MPR) allows the operator to cut three-dimensional (3D) echocardiographic data sets in infinite planes, and to review the moving image in three simultaneous orthogonal planes. We sought to describe the clinical utility of MPR of 3D echocardiography for analysis of congenitally malformed hearts. METHODS: Cross-sectional and 3D MPR echocardiography was performed in 300 patients with congenitally malformed hearts. RESULTS: Analysis in multiplanar mode was possible in all patients. New, clinically important information, which altered management or changed the principal diagnosis, was obtained in 32 (11%) cases. This determined suitability for biventricular repair in 11 patients, clarified the morphology of atrioventricular valves in 7, helped in assessment of aortic, mitral, or prosthetic valvar disease in 13, and identified a vascular ring in the other patient. CONCLUSIONS: 3D MPR is feasible in the setting of the congenitally malformed heart, permitting focused and in-depth analysis. This substantially improves the understanding of functional morphology, above the information derived from cross-sectional echocardiography. We recommend the use of the 3D format with MPR for patients with complex congenital cardiac disease.

A multiplanar three dimensional echocardiographic study of mitral valvar annular function in children with normal and regurgitant valves.

INTRODUCTION: The mitral valvar complex is difficult to visualise accurately in only two dimensions. Three-dimensional echocardiography gives new insight into the dynamic changes of intra-cardiac structures during the cardiac cycle. The aim of this study was to study the mitral annulus in systole and diastole in normal children using three-dimensional echocardiography, and to analyse the effect of regurgitation on annular function. MATERIALS AND METHODS: Three-dimensional echocardiographic datasets, acquired in 11 consecutive subjects with mitral regurgitation, and 20 normal subjects, were analysed offline using simultaneous multiplanar review. RESULTS: The mitral valvar annular area decreased in diastole, and increased in systole, in both groups. The annulus in patients with mitral regurgitation is dilated compared to normal subjects, the systolic value for those with regurgitation having a mean of 6.79 plus or minus 2.55 centimetres2/metres2, and the diastolic value a mean of 5.01 plus or minus 1.78 centimetres2/metres2, as opposed to a systolic mean value of 5.28 centimetres2/metres2 plus or minus 1.68, p = 0.091, and diastolic mean value of 3.05 centimetres2/metres2 plus or minus 0.90, in normal subjects (p less than 0.0001). The proportional change in mitral valvar annular area from systole to diastole showed a trend towards being smaller in those with mitral regurgitation, although this did not reach significance (24.8% versus 41.13%, p equal to 0.249). Analysis of subgroups of patients with moderate or severe mitral regurgitation showed mitral excursion, expressed as percentage of left ventricular length, to be significantly less than in normal subjects, at 12.78 plus or minus 5.10% versus 15.84 plus or minus 4.23% (p equal to 0.012). CONCLUSIONS: Mitral valvar annular area in children decreases in diastole, and increases in systole. In those with mitral regurgitation, the annulus is dilated and the dynamic annular function is depressed.

Assessment of the evolution of normal fetal diastolic function during mid and late gestation by spectral Doppler tissue echocardiography.

OBJECTIVE: To establish gestational age-specific reference values in healthy singleton fetuses, we prospectively assessed the evolution of diastolic longitudinal wall-motion velocities by spectral Doppler tissue imaging. METHODS: Early (Ea) and late diastolic (Aa) peak Doppler tissue imaging velocities were analyzed in 114 fetuses (age range: 14-42 weeks) at the base of right ventricular free wall, ventricular septum, and left ventricular free wall and compared with early (E) and late (A) diastolic peak Doppler inflow velocities. RESULTS: A linear increase in Ea, Aa, and Ea/Aa ratio was documented at all sites with advancing gestation. Likewise, the peak E flow velocities of both atrioventricular valves and the tricuspid peak A flow velocity increased. The ratio of peak E/Ea velocities decreased exponentially as a result of a more rapid increase in Ea than E, to reach a stable E/Ea relationship only in the early third trimester. CONCLUSIONS: There was a strong positive correlation between Ea and Aa velocities and gestational age indicating improved diastolic myocardial lengthening with advancing gestation. Reference charts for Doppler tissue imaging velocities were established that will allow identification of fetal diastolic function abnormalities.

Three-dimensional echocardiography improves the understanding of the mechanisms and site of left atrioventricular valve regurgitation in atrioventricular septal defect.

OBJECTIVES: The purpose of this study was to determine whether 3-dimensional echocardiography (3DE) provides additional information regarding the mechanisms and sites of left atrioventricular valve regurgitation in atrioventricular septal defect compared with transesophageal 2-dimensional echocardiography (2DE). METHODS: Eleven patients with a median age of 5.4 years (2.9-11.6 years) and a median weight of 16.8 kg (13.7-38.3 kg) with an atrioventricular septal defect underwent simultaneous transesophageal 2DE and 3DE before operation. RESULTS: The 2DE-3DE agreement for the assessment of the superior and mural leaflet size was 72.7%. The 2DE-3DE agreement for coaptation failure, a residual or primary cleft, and commissural abnormalities as a mechanism of regurgitation were 72.7%, 63.6%, and 36.4%, respectively. For jet sites the 2DE-3DE agreement was 63.6% for a commissural and central location. CONCLUSION: Three-dimensional echocardiography provides new and superior data regarding the mechanisms and sites of left atrioventricular valve regurgitation in atrioventricular septal defect.

The impact of patch augmentation on left atrioventricular valve dynamics in patients with atrioventricular septal defects: early and midterm follow-up.

OBJECTIVE: Left atrioventricular valve pericardial patch may prevent valve replacement. We assessed patch annular dynamics compared with conventional repair and normal annuli. METHODS: Transesophageal 3-dimensional echocardiography was acquired preoperatively and postoperatively in atrioventricular septal defects (n = 10, 5 patch, 5 conventional repair). Real-time 3-dimensional annular motion at midterm was compared with that of healthy children (n = 10). Parameters were: annular area, perimeter, segmental diameter, bending angle, stenosis, and regurgitation. RESULTS: Regurgitant jet area ratio decreased in both patient groups. Conventional repair reduced annular area (P = .02). Patch repair showed an annular area larger than normal (P = .01). Control subjects had increased systolic area whereas operative groups showed a reduction. Patch repair had segmental diameters similar to normal whereas conventional repair was inhomogeneous. Annular bending angle was maintained after operation. CONCLUSION: Patch repair in pediatrics shows durability without shrinkage or expansion. Improved stenosis and regurgitation does not change by midterm. Operation causes increased annular stiffness and diminished compliance. Neither technique establishes normal annular eccentricity.

Three-dimensional tricuspid annular function provides insight into the mechanisms of tricuspid valve regurgitation in classic hypoplastic left heart syndrome.

BACKGROUND: Tricuspid regurgitation (TR) has a negative impact on outcome in hypoplastic left heart syndrome (HLHS). There is a paucity of data assessing the mechanisms of TR. Lateral forces from the left ventricle play an important role in normal tricuspid valve (TV) function. This study evaluates the role of real-time 3-dimensional echocardiography in the assessment of the TV annulus and subvalvular apparatus in HLHS. METHODS: In all, 31 real-time 3-dimensional echocardiographic studies from 26 patients (1 day-17 years old) with classic HLHS were included. In all, 7 studies were before and 8 were after first-stage palliation; 8 were postcavopulmonary shunt and 8 were post-Fontan. Only patients with severe left ventricular attenuation were included. Studies were subdivided into two groups: mild TR (N = 20) and severe TR (STR) (N = 11). Data from 17 control subjects were used for comparison. TV annular area, bending angle (saddle shape), and position of anterior papillary muscle were analyzed. RESULTS: All annular segments moved in equally during systole in mild TR, whereas septal-lateral diameter contraction was significantly diminished in STR. TV annular area was larger in HLHS (mild TR, 1265 +/- 325; STR, 1767 +/- 450 mm2/m2) than in control subjects (762 +/- 85) (P < .001). However, area change was reduced only in STR (12.4 +/- 4.6% vs control subjects 20.4 +/- 8.3%, P < .05). Annular shape was flat during systole in HLHS. Anterior papillary muscle was laterally displaced in STR. CONCLUSIONS: TV annular dynamics were different in patients with HLHS as a result of lack of interaction form left ventricle. Annular function, especially in septal-lateral direction, was further impaired in STR.