Comparison of right ventricular deformation and dyssynchrony in patients with different subtypes of hypoplastic left heart syndrome after Fontan surgery using two-dimensional speckle tracking.

BACKGROUND: The left ventricle in patients with hypoplastic left heart syndrome may influence right ventricular function and outcome. We aimed to investigate differences in right ventricular deformation and intraventricular dyssynchrony between hypoplastic left heart syndrome patients with different anatomical subtypes and left ventricle sizes after Fontan surgery using two-dimensional speckle tracking. PATIENTS AND METHODS: We examined 29 hypoplastic left heart syndrome patients aged 5.4 plus or minus 2.8 years after Fontan surgery and compared 15 patients with mitral and aortic atresia with the remaining 14 patients with other anatomic subtypes. We used two-dimensional speckle tracking to measure the global and regional systolic longitudinal strain and strain rate as well as intraventricular dyssynchrony. RESULTS: Global strain (-19.5, 2.8% versus -17.4, 3.9%) and global strain rate (-1.0, 0.2 per second versus -0.9, 0.3 per second) were not different between groups. The mitral and aortic atresia group had higher strain in the basal septal (-13.0, 5.0% versus -3.9, 9.3%, p = 0.003) and mid-septal (-19.4, 4.7% versus -13.2, 6.5%, p = 0.009) segments, and higher strain rates in the mid-septal segment (-1.14, 0.3 per second versus -0.95, 0.4 per second, p = 0.047), smaller left ventricle area (0.18, 0.41 square centimetre versus 2.83, 2.07 square centimetre, p = 0.0001), and shorter wall-to-wall delay (38, 29 milliseconds versus 81, 57 milliseconds, p = 0.02). CONCLUSION: Significant differences in regional deformation and intraventricular dyssynchrony exist between the mitral and aortic atresia subtype with small left ventricles and the other anatomic subtypes with larger left ventricles after Fontan surgery.

Impact of afterload reduction strategies on regional tissue oxygenation after the Norwood procedure for hypoplastic left heart syndrome.

OBJECTIVES: Low cerebral tissue oxygenation saturations have been observed by near-infrared spectroscopy (NIRS) after the Norwood procedure. Altered cerebral vascular resistance and pharmacological afterload reduction redirecting blood flow away from the cerebral circulation are possible mechanisms. METHODS: Two different afterload reduction strategies were evaluated in patients with hypoplastic left heart syndrome or variants after the Norwood procedure. In patients of Group 1 (n=34), afterload reduction was controlled with sodium nitroprusside or with the α-blocker phentolamine. In addition, a phosphodiesterase-III inhibitor was administered. Patients of Group 2 (n=34) received a phosphodiesterase-III inhibitor only. Cerebral and somatic tissue oxygenation saturations and routine intensive care monitoring data were recorded for 24 h before and 48 h after the Norwood procedure. Mean values of the last 4 preoperative (baseline) and of the first and last 4 postoperative hours (early and late course) were calculated. RESULTS: Baseline, early and late cerebral saturations were 58±7, 52±9 and 60±6% for Group 1 and 58±7, 52±12 and 61±7% for Group 2 and somatic saturations were 59±8, 76±10 and 67±9% and 58±9, 78±8 and 69±10%, respectively. Regional saturations were not different between groups. The postoperative cerebral tissue oxygen saturation was below 40% for 50 (0-1040) min in Group 1 and for 45 (0-720) min in Group 2 (P=1.00). Preoperative cerebral NIRS values (OR 0.85 [0.76-0.96], P=0.007), age at operation (OR 1.39 [1.02-1.88], P=0.034) and early postoperative diastolic blood pressure (OR 0.88 [0.78-0.99], P=0.038) were associated with cerebral tissue oxygen saturations below 40% for more than 60 min. Patients with a prolonged period of low cerebral tissue oxygen saturation had longer duration of mechanical ventilation (69 (37-192) vs 60 (33-238) h, P=0.039) and afterload reduction therapy was terminated later (95 (47-696) vs 74 (39-650) h, P=0.006). Early mortality was 9.4% (3 of 32) compared with 2.8% (1 of 36) in the remainder (P=0.336). CONCLUSIONS: The postoperative decline of cerebral tissue oxygen saturation was observed with both afterload reduction strategies. The difference between cerebral and somatic NIRS values may indicate a mismatch between cerebral and splanchnic oxygenation. Other strategies to improve cerebral tissue oxygenation are warranted.

Two-dimensional global longitudinal strain rate is a preload independent index of systemic right ventricular contractility in hypoplastic left heart syndrome patients after Fontan operation.

BACKGROUND: Assessment of systemic right ventricular (RV) function in patients with hypoplastic left heart syndrome is important during long-term follow-up after Fontan repair. Traditional echocardiographic parameters to evaluate systolic ventricular function are affected by loading conditions. The only generally accepted load-independent parameter of systolic function, end systolic elastance (Ees), requires invasive catheterization. Therefore, we sought to determine if parameters obtained by 2-dimensional speckle tracking (2DST) were affected by acute changes in preload and correlated with catheterization-derived indices of RV contractility in hypoplastic left heart syndrome patients after Fontan palliation. METHODS AND RESULTS: Fifty-two patients with hypoplastic left heart syndrome (median age, 6.6; range 2.9-22.2 years) were prospectively enrolled to have echocardiography and conductance catheter studies performed simultaneously. We compared traditional echo, 2-dimensional speckle tracking and catheterization-derived parameters during different states of preload at baseline and during dobutamine infusion. Global longitudinal strain (S) showed a tendency to decrease with preload reduction, whereas global longitudinal strain rate (SR) did not change (S: -17.7 ± 3.4% versus -16.9 ± 3.8%, P=0.08; SR: -1.30 ± 0.29 versus -1.34 ± 0.34 s(-1), P=0.3). S did not change with dobutamine infusion (-17.7 ± 3.4% versus -18.4 ± 3.9%, P=0.24), whereas SR increased significantly (-1.30 ± 0.29 versus -2.26 ± 0.49 s(-1), P<0.001). RV Ees correlated with SR (rs= -0.47, P<0.001), but not with S (rs=0.07, P=0.5) or other echocardiographic parameters. CONCLUSIONS: In contrast to S, SR was not affected by preload and correlated with Ees of the systemic RV. SR may be a useful noninvasive surrogate of RV contractility and suitable for follow-up of patients with hypoplastic left heart syndrome after Fontan palliation.

Monitoring of regional tissue oxygenation with near-infrared spectroscopy during the early postoperative course after superior cavopulmonary anastomosis.

OBJECTIVES: Near-infrared spectroscopy (NIRS) offers continuous non-invasive monitoring of regional tissue oxygenation. We evaluated NIRS monitoring during the postoperative course after superior cavopulmonary anastomosis in patients with hypoplastic left heart syndrome and anatomically related malformations. METHODS: Cerebral (cSO(2)) and somatic (sSO(2)) tissue oxygenations were recorded for 48 h and compared with routine measures of intensive care monitoring. Changes in parameters in the case of postoperative complications were evaluated. RESULTS: Data were obtained from 32 patients. Median age at operation was 2.9 (1.5-10.0) months and weight was 5.3 ± 1.0 kg. Postoperative complications occurred in 7 patients (pulmonary artery thrombus n = 4, pneumothorax n = 1, cardiopulmonary resuscitation n = 1 and low-cardiac output n = 1). cSO(2) was 44 ± 14% at the end of the operation and reached its minimum of 40 ± 11% 2 h later (P = 0.018). Overall, cSO(2) was depressed early after surgery and increased from a mean of 42 ± 11% during the first 4 postoperative hours to 57 ± 8% in the last 4 h of the study period (P < 0.001). The sSO(2) decreased from 77 ± 11% during the early postoperative course to 68 ± 9% within the later course (P < 0.001). The cSO(2) correlated with the arterial partial pressure of oxygen (pO(2), r = 0.364, P < 0.001), with the arterial oxygen saturation (SaO(2), r = 0.547, P < 0.001) and with the central venous oxygen saturation providing the strongest correlation (SvO(2), r = 0.686, P < 0.001). Analysis of agreement between cSO(2) and SvO(2) measurements revealed a mean bias of 0.97 with limits of agreement between 19.8 and -17.9%. Inclusion of both cSO(2) and sSO(2) into a linear regression model slightly improved the prediction of SvO(2) from NIRS values (r = 0.706, P < 0.001). The mean values of cSO(2), sSO(2), SaO(2) and SvO(2) during the early postoperative period were lower in patients with complications (cSO(2): 45 ± 9 vs 29 ± 5%, P < 0.001; sSO(2): 80 ± 11 vs 70 ± 6%, P = 0.004; SaO(2): 76 ± 8 vs 66 ± 6%, P = 0.004; SvO(2): 48 ± 14 vs 32 ± 6%, P < 0.001). CONCLUSIONS: NIRS technology allows inferring the global oxygenation from continuous non-invasive measurements of regional tissue oxygenation. The cSO(2) is lowered in the early postoperative course. Lower cSO(2) values in the early postoperative course may be predictive of postoperative complications.

Arterial elastance and its impact on intrinsic right ventricular function in palliated hypoplastic left heart syndrome.

BACKGROUND: Systemic right ventricular (RV) function is a major determinant of long-term outcome in patients with palliated hypoplastic left heart syndrome (HLHS). Abnormal elastic properties of the reconstructed aorta may negatively impact on ventricular function. We therefore aimed to assess arterial elastance and its relationship to systemic RV function in these patients. METHODS: Fifty-six HLHS patients (median age 5.4 years; range 2.9-14.2 years) were studied at a median of 2.6 years (range 0.8-12.7 years) after completion of the Fontan circulation with the pressure-volume conductance system. RESULTS: Arterial elastance (Ea) was abnormally high and correlated inversely with RV ejection fraction (r=-0.42, P=0.001). However, end systolic elastance (Ees) - a load independent measure of intrinsic systolic ventricular function - and more so end diastolic stiffness (Eed) were positively correlated with Ea (Ees vs. Ea: r=0.44, P=0.001: Eed vs. Ea: r=0.62, P<0.0001). Patients who were treated for significant aortic arch obstruction after surgical palliation showed higher Ea and Eed even four years after successful treatment compared to the remainder of the group (Ea: 3.4 ± 1.2 vs. 2.8 ± 1.0 mmHg/ml, P=0.04 and Eed: 0.67 ± 0.44 vs. 0.45 ± 0.3 mmHg/ml, P=0.04). CONCLUSIONS: Arterial elastance is abnormally high in palliated HLHS patients and negatively impacts on ejection fraction but not on intrinsic systolic RV function early after completion of the Fontan circulation. Increased arterial elastance, however, is associated with increased RV diastolic stiffness with potential adverse effects on long-term outcome. Furthermore, arterial elastance and diastolic stiffness are particularly high in patients who needed treatment for aortic arch obstruction.

Does left ventricular size impact on intrinsic right ventricular function in hypoplastic left heart syndrome?

BACKGROUND: The size of the remnant left ventricle (LV) may influence right ventricular function and thus long-term outcome in palliated hypoplastic left heart syndrome (HLHS). We therefore sought to assess the impact of the size of the hypoplastic LV on intrinsic RV function in HLHS patients after Fontan surgery. METHODS: Fifty-seven HLHS patients were studied 2.5 (range: 0.8-12.6) years after Fontan-type palliation with the pressure-volume conductance system. The patient cohort was divided into two groups according to the median LV area index (group 1: LV area index ≤ 1.33 cm(2)/m(2), n=29; group 2: LV area index>1.33 cm(2)/m(2), n=28). RESULTS: The slopes of the end systolic elastance (Ees) and the preload recruitable stroke work relation (Mw) were not different between group 1 and 2 (Ees: 2.70 ± 1.92 vs. 3.68 ± 2.68 mmHg/ml; Mw: 52.75 ± 14.98 vs. 51.09 ± 16.63 mmHg x ml; P=NS for all). Furthermore, the systolic responses to dobutamine were not statistically different between groups. However, the slope of the end diastolic stiffness (Eed) was higher in group 2 and catecholaminergic stimulation resulted in a decrease in Eed in group 2 (group 1: 0.40 ± 0.26 vs. 0.52 ± 0.45; group 2: 0.68 ± 0.44 vs. 0.47 ± 0.38 mmHg/ml, P<0.01). Furthermore Eed was lowest in patients with mitral atresia/aortic atresia, the anatomic subgroup with the smallest LV remnant. CONCLUSIONS: Intrinsic systolic RV function is not affected by the size of the hypoplastic LV in survivors of surgical palliation of HLHS. Diastolic stiffness, however, was higher in patients with a larger LV remnant.

Can we use the end systolic volume index to monitor intrinsic right ventricular function after repair of tetralogy of Fallot?

BACKGROUND: After tetralogy of Fallot (ToF) repair the right ventricle (RV) is commonly exposed to abnormal volume load resulting from pulmonary regurgitation (PR) leading to progressive RV dilatation. The objective of this study was to assess the relationship between RV volumes, especially the end systolic volume index (ESVi), and RV contractility in patients after ToF repair and significant PR and to determine whether RV dilatation reflects intrinsic RV dysfunction in these patients. METHODS: Twenty-nine ToF patients were studied 11.6 (range: 1.9-30.1) years after repair with the pressure-volume conductance system. The patient cohort was divided into two groups according to the median ESVi (group 1: ESVi<34.7 ml/m(2)(×1.18), n = 14; group 2: ESVi ≥ 34.7 ml/m(2)(×1.18), n = 15). RESULTS: The slope of the end systolic pressure-volume relationship (end systolic elastance, Ees) was higher in group 1 compared to group 2 both at baseline and during dobutamine infusion (0.87 ± 0.36 vs. 0.46 ± 0.28 mm Hg/ml and 1.50 ± 0.77 vs. 0.92 ± 0.37 mm Hg/ml; P<0.005 and P = 0.02, respectively). Overall, Ees at baseline correlated significantly with ESVi and also with the end diastolic volume index (r = -0.64, and P<0.001 for both). Receiver operating characteristic curve analysis revealed that ESVi was superior to RV ejection fraction (EF) in predicting an Ees in the lowest quartile of the study group (area under curve ESVi vs. EF: 0.84 (0.64-0.95) vs. 0.68 (0.47-0.85); P = 0.015). CONCLUSION: ESVi is a valid estimate of intrinsic RV function in repaired ToF patients with residual PR and in this respect seems superior to EF. These data underscore the importance of serial ventricular volume assessment in the follow-up of these patients.

Interventional creation of an atrial septal defect and its impact on right ventricular function: an animal study with the pressure-volume conductance system.

BACKGROUND: The aim of our study was to assess the suitability of different interventional techniques to create an atrial septal defect (ASD) and to evaluate the short-term effects of right ventricular (RV) volume overload on RV contractility in the growing swine. METHODS: Thirteen ASD and six control animals were studied. An ASD was created by balloon dilatation (BD) of the fossa ovalis (n = 4) or by implantation of a multi-perforated Amplatzer Septal Occluder (n = 4) or a patch-less nitinol device (n = 5). After 4.8 (3.9-6.0) weeks, the amounts of left-to-right shunting (Qp/Qs) and RV contractility (end systolic elastance - Ees) were assessed. RESULTS: In the ASD group, a significant left-to-right shunt could be documented (Qp/Qs 1.5 ± ± 0.4). However, a shunt was absent in the BD subgroup (Qp/Qs 1.1 ± 0.1). In animals with devices implanted, a significant relationship between the post-mortem ASD area and Qp/Qs was found (r = 0.68, p < 0.05). Compared to controls, RV contractility was not significantly impaired at rest and during dobutamine in ASD animals (Ees: 0.40 ± 0.20 vs 0.54 ± 0.12 and 0.75 ± 0.29 vs 1.04 ± 0.24 mm Hg/mL, p = NS for both). CONCLUSIONS: Device implantation is necessary to create a patent ASD resulting in significant left-to-right shunting. In an experimental ASD model, a five week period of chronic RV volume overload does not alter RV contractility significantly.