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PubMed2006

Plasma brain natriuretic peptide levels, right ventricular volume overload and exercise capacity in adolescents after surgical repair of tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) volume overload secondary to pulmonary regurgitation contributes to long-term morbidities in patients after tetralogy of Fallot (TOF) repair. We tested the hypothesis that plasma brain natriuretic peptide (BNP) levels relate to RV volume overload, pulmonary regurgitation, and exercise capacity in adolescents after TOF repair. METHODS: We assessed the RV function echocardiographically and plasma BNP levels in 32 postoperative TOF patients aged 14.7+/-3.1 years and 20 age-matched controls. Eighteen patients further underwent cardiovascular magnetic resonance imaging and 26 had exercise testing. RESULTS: Compared with controls, patients had significantly higher BNP levels (p=0.027), greater indexed RV end-diastolic dimension (p<0.001), increased RV myocardial performance index (p=0.005), and reduced tricuspid annular systolic velocity (p=0.008). Multivariate analysis identified indexed RV end-diastolic dimension as the only significant determinant of plasma BNP levels (beta=0.69, p<0.001). Plasma BNP levels correlated positively with indexed RV end-diastolic volume (r=0.6, p=0.009) and pulmonary regurgitant fraction (r=0.54, p=0.026), and negatively with exercise duration (r=-0.45, p=0.021), peak oxygen consumption (r=-0.43, p=0.03), and minute ventilation at maximal exercise (r=-0.52, p=0.006). Multivariate analysis demonstrated BNP levels (beta=-0.43, p=0.034) and body mass index (beta=-0.40, p=0.036) to be independent predictors of peak oxygen consumption. No relations were found between BNP levels and RV myocardial performance index, tricuspid annular velocities and RV ejection fraction. CONCLUSIONS: In adolescent patients after TOF repair, plasma BNP levels relate to RV volume overload, pulmonary regurgitation and exercise capacity.

Adolescent↗
PubMed1998

Effect of oxygen on pulmonary hemodynamics and incidence of atrial fibrillation after noncardiac thoracotomy.

OBJECTIVE: The mechanism of postthoracotomy atrial fibrillation (AF) could be related to right ventricular (RV) strain. The effect of oxygen on the occurrence of postoperative AF and on RV function was studied. DESIGN: A prospective, randomized study. SETTING: A university hospital. PARTICIPANTS: Twenty-four noncardiac thoracotomy patients. INTERVENTIONS: At the end of the postoperative anesthesia care unit period, the patients were randomly allocated to receive 35% oxygen until either the third (P = prolonged group) or the first postoperative morning (S = short group). MEASUREMENTS AND MAIN RESULTS: Measurement of hemodynamic variables using a thermodilution pulmonary artery catheter, oxygenation, concentration of plasma atrial natriuretic peptide (ANP) and Holter monitoring were started preoperatively and continued for the 3 postoperative days (PODs). Systolic RV pressure (systolic RVP) and pulmonary vascular resistance (PVR) increased postoperatively only in group S. Major changes in RV performance were not seen with echocardiography or the thermodilution method in any patient. Silent episodes of AF occurred in three patients (25%) in group P and in one patient (8%) in group S (not significant [NS]) without deviations in plasma ANP concentration. On each of the 3 PODs, all patients were exposed to 60% oxygen for 15 minutes. Systolic RVP decreased significantly during the exposure to 60% oxygen only in group S, but not in patients developing AF. Predictive factors for AF were a high preoperative PVR, intraoperative bleeding necessitating volume loading, and elevated systolic RVP immediately after thoracotomy. CONCLUSION: Short episodes of AF occurred irrespective of the length of oxygen therapy. Occurrence of AF could not be explained by changes in RV function.

Atrial Fibrillation↗
PubMed2003

Use of bovine jugular vein to reconstruct the right ventricular outflow tract: early results.

OBJECTIVE: We evaluate early results of bovine jugular vein conduits in the pulmonary outflow. METHODS: Between April 2000 and September 2001, 31 conduits were placed in the outflow of the right ventricle. Patients who received a conduit as a staged surgical procedure were excluded (n = 3). Implantation age ranged from 0 to 21 years (median, 3.4 years). Conduit diameter ranged from 12 to 20 mm (median, 14 mm). Transthoracic echocardiography was performed at discharge and 3 months after surgery. Patients with significant pulmonary regurgitation and/or stenosis underwent cardiac catheterization. RESULTS: Four patients died during the follow-up period. Three deaths were unrelated to the conduit. One death was related to the complete thrombosis of the conduit. At 3 months evaluation, pulmonary valve regurgitation was absent or trivial in 19, mild in 2 and severe in 3 of 24 survivors. Four patients had nonfatal conduit-related complications. A transient thrombus formation within 1 leaflet was noted postoperatively in a patient with a moderate pulmonary regurgitation. Three patients required reoperation 3 to 5.8 months after the implantation for conduit failure (mean, 4.3 months). Cardiac catheterization before replacement revealed an aneurysmal dilation of the conduit below a severe stenosis of the pulmonary bifurcation due to important neointimal proliferation. CONCLUSIONS: Early failure of bovine jugular vein valved conduits can occur because of exaggerated intimal proliferation or thrombotic process within the conduit. Because of these complications, close echocardiographic follow-up is mandatory during the first weeks after implantation.

Adolescent↗
PubMed2006

Reduction of right ventricular pacing in patients with dual-chamber ICDs.

BACKGROUND: Unnecessary right ventricular (RV) pacing in patients with implantable cardioverter defibrillators (ICD) may adversely affect heart failure morbidity and total mortality. Inhibition of Unnecessary RV Pacing with AV Search Hysteresis in ICDs (INTRINSIC RV) is a prospective, multicenter, randomized trial evaluating outcomes in ICD recipients programmed to single-chamber pacing (VVI) versus dual-chamber (DDDR) pacing with AV search hysteresis (AVSH). METHODS: Patients underwent ICD implant (for standard indications). The ICD was programmed to DDDR with AVSH regardless of any need for pacing. Rate-adaptive pacing was set at 60-130 ppm with dynamic AV delay from 200 to 90 ms. AVSH was programmed to search every 32 intervals and extend the AV delay by 50%. One week post-implant patients with ICDs were interrogated to assess the percentage of RV pacing with the expectation that most would have <20% RV pacing and would be randomized into INTRINSIC RV. Early analysis showed that targets for randomization were not met. AVSH parameters were modified under a protocol amendment to increase AV delay extension to 100%. We report findings related to this programming change based upon analyses of (nonrandomized) data pre- and post-amendment. RESULTS: Twenty-one percent of patients (n = 314) were enrolled pre-amendment and 79% (n = 1,216) were enrolled post-amendment. The mean percentage of RV pacing at the 1-week visit was 41.4 +/- 29.6% pre-amendment and 14.7 +/- 22.6% post-amendment (P < 0.0001). The proportion of patients eligible for randomization (RV pacing <20% at the 1-week visit) was 31.2% pre-amendment and 76.8% post-amendment (P < 0.0001). CONCLUSION: AVSH can dramatically reduce the percentage of RV pacing among ICD recipients.

Aged↗
PubMed2004

Magnetic resonance imaging analysis of right ventricular pressure-volume loops: in vivo validation and clinical application in patients with pulmonary hypertension.

BACKGROUND: The aims of this study were to validate MRI-derived right ventricular (RV) pressure-volume loops for assessment of RV myocardial contractility and then to apply this technique in patients with chronic RV pressure overload for assessment of myocardial contractility, ventricular pump function, and VA coupling. METHODS AND RESULTS: Flow-directed catheters were guided under MR fluoroscopy (1.5 T) into the RV for invasive pressure measurements. Simultaneously, ventricular volumes and myocardial mass were assessed from cine MRI. From sampled data, RV pressure-volume loops were constructed, and maximal ventricular elastance indexed to myocardial mass (E(max_i)) was derived by use of a single-beat estimation method. This MRI method was first validated in vivo (6 swine), with conductance techniques used as reference. Bland-Altman test showed good agreement between methods (E(max_i)=5.1+/-0.5 versus 5.8+/-0.7 mm Hg x mL(-1) x 100 g(-1), respectively; P=0.08). Subsequently, the MRI method was applied in 12 subjects: 6 control subjects and 6 patients with chronic RV pressure overload from pulmonary hypertension. In these patients, indexes of RV pump function (cardiac index), E(max_i), and VA coupling (E(max)/E(a)) were assessed. In patients with pulmonary hypertension, RV pump function was decreased (cardiac index, 2.2+/-0.5 versus 2.9+/-0.4 L x min(-1) x m(-2); P<0.01), myocardial contractility was enhanced (E(max_I), 9.2+/-1.1 versus 5.0+/-0.9 mm Hg x mL(-1) x 100 g(-1); P<0.01), and VA coupling was inefficient (E(max)/E(a), 1.1+/-0.3 versus 1.9+/-0.4; P<0.01) compared with control subjects. CONCLUSIONS: RV myocardial contractility can be determined from MRI-derived pressure-volume loops. Chronic RV pressure overload was associated with reduced RV pump function despite enhanced RV myocardial contractility. The proposed MRI approach is a promising tool to assess RV contractility in the clinical setting.

Adolescent↗
PubMed2001

RV function in stable and unstable VT: is there a need for hemodynamic monitoring in future defibrillators?

During electrophysiological investigation of 22 patients with VT or aborted sudden cardiac death, arterial and RV pressures were measured. The time courses of mean arterial pressure (MAP), RV pulse pressure (RVPP), RV pulse pressure integral (RVPPI), and maximum right ventricular dP/dt (RV dP/dtmax) were followed during the first 15 seconds after VT induction. Compared to basal (preinduction) conditions, the RVPPI decreased by 41+/-10% (mean +/- SD) after 10-15 seconds of VT in 11 patients with stable VT and by 75+/-8% in 11 patients with unstable VT (MAP < 60 mmHg 15 s after VT onset). RVPP decreased by 13+/-11% after 10-15 seconds of VT in the stable VT group and by 50+/-16% in the unstable VT group. For RV dP/dtmax, these decreases were 4+/-22% in the stable VT group and 37+/-24% in the unstable VT group. There was a good correlation between percent decrease in MAP and percent decrease in RVPPI, RVPP, and RV dP/dtmax at 5-10 seconds (r = 0.86, 0.81, and 0.73, respectively) and 10-15 seconds (r = 0.84, 0.82, and 0.69, respectively) after VT onset. There was hardly any overlap of distributions of the individual values with the RVPPI parameter between the two VT groups. Comparing and correlating the percent decrease in mean arterial pressure with the RVPPI, RVPP, and RV dP/dtmax during induced VT, RVPPI demonstrated the most significant and specific changes in discriminating stable from unstable rhythms. However, by comparing RVPPI and RVPP using the area under the receiver operating characteristic curves, there was no significant statistical difference between the two parameters. By integrating rate criteria, electrogram signal analysis, and RVPPI or RVPP as a hemodynamic criterion, detection and treatment algorithms could improve the performance of future implantable defibrillators and avoiding shocks in VTs that can be terminated by antitachycardia pacing.

Aged↗
PubMed2006

Inferior wall myocardial infarction with or without right ventricular involvement--treatment and in-hospital course.

INTRODUCTION: Right ventricular infarction (RVI) is most commonly associated with inferior wall infarction (20-50% of cases). Clinical presentation of RVI may vary. AIM: Assessment of outcome and clinical course of myocardial infarction in patients with inferior wall myocardial infarction with or without RVI. Additionally, risk stratification was attempted in the above-mentioned groups of patients. METHODS: The analysis involved 181 consecutive patients (pts) with inferior wall myocardial infarction hospitalised between 1 July 2000 and 1 July 2002. RESULTS: Nineteen in-hospital deaths were noted in the study group (mortality 10.5%), reinfarction occurred in 6 (3.3%) pts, ischaemic stroke in 1 (0.6%) patient, and 2 (1.1%) pts had transient ischaemic attack. Cardiogenic shock occurred in 20 (11.0%) pts , ventricular fibrillation in 15 (8.3%) pts, and pulmonary oedema in 9 (4.9%) pts. In the subgroup of 161 pts without cardiogenic shock 8 (4.9%) pts died. Thrombolytic therapy was administered in 96 (53%) subjects. Isolated inferior wall myocardial infarction was diagnosed in 94 (51.9%) of 181 pts and RVI in 65 (35.9%) pts. Mortality rate in the RVI group was significantly higher than in inferior wall myocardial infarction without RVI and was 18.5% vs 2.12% (p=0.0003), respectively (excluding patients with cardiogenic shock: 11.1% vs 1.2%, respectively; p=0.016). In patients with RVI aged above 70 years, the mortality rate was significantly higher than in younger patients (32% vs 10%, p=0.002). In a subgroup with RVI treated with thrombolysis mortality was considerably higher in subjects aged >70 years compared to patients below 70 years (38.5% vs 7.7%, p=0.017). CONCLUSIONS: RVI is associated with worse prognosis and increased number of in-hospital complications. Older patients aged >70 years have definitely poorer prognosis. Thrombolytic therapy in a subgroup of older patients with RVI remains ineffective.

Age Factors↗
PubMed2005

Angiocardiographic pressure volume loops in the analysis of right ventricular function after repair of tetralogy of Fallot.

OBJECTIVE: The objective of the study was to estimate the capacity of pressure volume (PV) loop analysis to assess right ventricular (RV) function after Fallot (TOF) repair. PATIENTS: Fifty six patients were examined after TOF repair. PV loops were constructed from RV angiocardiography and simultaneous pressure measurement. Patients were divided in three groups according to RV size and pressure (Group I: normal RV size and pressure; group II: enlarged RV, near normal pressure; group III: normal RV size, elevated pressure). MAIN OUTCOME MEASURES: Systolic stroke work corrected for body surface area (W/BSA) and for RV enddiastolic volume (W/EDV), peak RV filling (PFR) and emptying rates (PER) corrected for RV stroke volume, cycle efficiency (CE), RV ejection fraction (RVEF). RESULTS: W/BSA was significantly higher in group II than in group I (0.19 +/- 0.05 vs. 0.11 +/- 0.04 J/m(2), p < 0.001) and was similar between groups II and III (0.19 +/- 0.05 vs. 0.17 +/- 0.05 J/m(2) ; NS). W/EDV was similar in groups I and II (12.4 +/- 5.4 vs. 12.4 +/- 2.9 mmHg; NS). CE was smallest in group II. The difference was significant between groups II and III (0.62 +/- 0.08 vs. 0.73 +/- 0.09; p < 0.05). RVEF was negatively correlated to RV end systolic volume (RVESV) in the patients of groups I and II (r = -0.32, p < 0.05). A similar correlation was found between PFR and RVESV (r = -0.28, p < 0.05). CONCLUSIONS: Analysis of a single PV loop allows quantification of RV load after TOF repair. W/BSA is increased to the same extent under volume and pressure load. The lack of decrease in W/EDV in patients with enlarged RV indicates that RV is capable to perform adequate work in a wide range. RVESV is a useful measure for estimating RV function after TOF repair depicting parameters of systolic and diastolic RV function.

Adolescent↗
PubMed2004

Backscatter evaluation of myocardial functional and textural findings in children with right ventricular pressure and/or volume overload.

Integrated backscatter (IBS) analysis represents a recent echocardiographic technique for evaluating the textural and functional state of the right ventricular (RV) myocardium. We undertook our study to (1) define the potential impact of age or morphometric parameters (height, weight, and body surface area) on RV IBS indexes, and (2) compare RV IBS data among normal subjects and children with different conditions of RV overload. We studied 34 patients (mean age 14.6 +/- 4.7 years) divided into 3 groups: 14 patients with RV volume overload related to moderate to large atrial septal defect (group I); (2) 10 patients with RV pressure overload due to transposition of the great arteries after the Mustard procedure (group II); and (3) 10 patients with RV volume and pressure overload due to pulmonary regurgitation and stenosis after corrective surgery for tetralogy of Fallot (group III). In addition, 20 children with structurally normal hearts were enrolled as the control group. The 4 groups were comparable with regard to age and morphometric parameters. IBS parameters were assessed as the magnitude of cyclic variation, determined as the difference between peak and nadir IBS values and the averaged myocardial IBS intensity. In the control group and group I, IBS parameters did not change significantly with age, height, weight, and body surface area. In contrast, in groups II and III, a significant correlation was found between cyclic variation and age at the study (p = 0.021, r = -0.71, and p = 0.006, r = -0.79, respectively). Furthermore, compared with the control group, cyclic variation and IBS intensity were significantly different only in groups II (p = 0.01 and p = 0.006, respectively) and III (both p <0.0001) but not in group I (p = 0.23 and p = 0.38, respectively). The lowest values of cyclic variation and the highest values of intensity were detected in group III patients. Thus, our data suggest that (1) in normal subjects, there is no correlation between RV IBS indexes and age or any morphometric parameters, and (2) IBS analysis is able to noninvasively detect differences in myocardial functional and textural properties in the presence of different conditions of RV overload.

Adolescent↗
PubMed1995

Regional left ventricular systolic function in relation to the cavity geometry in patients with chronic right ventricular pressure overload. A three-dimensional tagged magnetic resonance imaging study.

BACKGROUND: Distortion of the left ventricular (LV) cavity in patients with right ventricular pressure overload (RVPO) is well known. However, no direct measurements of regional myocardial function in terms of myocardial shortening and wall thickening are available; therefore, exactly how RVPO disturbs LV regional performance remains unclear. By using three-dimensional (3D) tagged magnetic resonance imaging, we were able to measure regional systolic function directly. Our objective was to study the relation between the distortion of the LV circular shape and regional LV function. METHODS AND RESULTS: In nine patients with RVPO and six healthy volunteers, four parallel short-axis images (with 12 radial tags) and two mutually orthogonal long-axis images (with four parallel tags) were generated, and endocardial and epicardial borders were manually traced. By integration of the short- and long-axis images, 3D reconstruction of the LV tracking points from end diastole to end systole was obtained. Data from the midventricular two short-axis image slices were analyzed. These were then divided into anterior, lateral, posterior, and septal regions. Circumferential and longitudinal shortening were then calculated from the endocardial and epicardial tag intersection points. Wall thickness and thickening were calculated by the 3D volume-element approach. An eccentricity index (EI), the ratio of septum-to-free-wall to anteroposterior diameters, was used to describe the shape of the LV cavity. The regional curvature was also measured. The RVPO group was characterized by flattening of the septum and LV lateral wall, decreased EI reflecting the distorted LV shape, altered distribution of endocardial circumferential shortening, and preserved ejection fraction. Changes in EI closely correlated with the septal curvature. The EI was smaller at end systole, reflecting further shape distortion relative to end diastole. Reduced myocardial performance, as measured by wall thickening and circumferential and longitudinal shortening fractions, was observed for the septum. A reduction in endocardial circumferential shortening of the septal and lateral walls was directly related to the end-systolic EI. In addition, whereas for healthy subjects a linear relation between area ejection fraction and endocardial circumferential shortening was observed, in RVPO patients a curvilinear (quadratic) relation was observed. CONCLUSIONS: In patients with RVPO, compared with healthy subjects, the septal function was reduced, as evidenced by reduced thickening and shortening fractions. The distortion in LV cavity at end systole due to the flattening of the septum contributes to preserved systolic ventricular function and nonuniform distribution in endocardial circumferential shortening.

Adult↗
PubMed1998

Pulmonary embolism: relation between the degree of right ventricle overload and the extent of perfusion defects.

BACKGROUND: Inasmuch as the presence of right ventricle (RV) overload in patients with pulmonary embolism (PE) is associated with a bad prognosis, evaluation of RV function in PE is of importance. This study was done to establish if the degree of RV overload can be predicted from the extent of perfusion defects (PDf). METHODS: One hundred twenty-one consecutive patients with PE diagnosed by lung scintigraphy (LS) were examined by echocardiography Doppler (ED) immediately after diagnosis. PDf were graded visually in categories (LS score 1 = < or =20%, 2 = >20% of total lung area) and on a continuous scale (normal perfusion = 0, no perfusion = 1). The reproducibility of both methods was tested. RV wall motion was assessed on a four-point scale (0 = normal to 3 = severely hypokinetic). The distance from LV posterior wall to RV anterior wall and dimensions of RV and LV were measured. Pulmonary artery systolic pressure (PAsP) was calculated by using the maximum velocity of tricuspid regurgitation. RESULTS: There were 51 patients with LS score 1 and 70 (58%) with score 2. In comparison with patients with LS score 1, those with score 2 more often had RV hypokinesis 2+ or 3+ (n = 49 vs n = 16) (p < 0.001), larger RV (34 +/- 6 mm [22 to 48] vs 29 +/- 5 [17 to 38]) (SD [range]) (p < 0.001) and higher PAsP (51 +/- 13 mm Hg [21 to 83] vs 42 +/- 14 [20 to 81]) (p < 0.001). The variability in both groups was large. With continuous scaling, PDf averaged 0.3. This was also the value that best discriminated RV hypokinesis 2+ or 3+ in a receiver operating characteristic curve. However, the variability for this scan scoring method was SD 0.073, giving a 95% confidence limit of +/-0.15. CONCLUSION: There is a significant correlation between RV overload and PDf, but the variability is large; therefore, an estimate of the size of perfusion defects in LS cannot replace ED in the assessment of PAsP and the degree of RV overload in PE.

Aged↗
PubMed2007

Effectiveness and cost-effectiveness of thrombolysis in submassive pulmonary embolism.

BACKGROUND: Thrombolytic therapy is controversial in patients with submassive pulmonary embolism. METHODS: We performed a cost-effectiveness analysis to compare health effects and costs of treatment with alteplase plus heparin sodium vs heparin alone in hemodynamically stable patients with pulmonary embolism and right ventricular dysfunction by developing a Markov model and using data from clinical trials and administrative sources. RESULTS: Based on data from a recent randomized trial, we assumed that the risk of clinical deterioration requiring treatment escalation was almost 3 times higher in patients who received heparin alone (23.2% vs 7.6%) but that the risk of death was equal in the 2 cohorts (2.7%). Based on registry data, we assumed that the risk of intracranial hemorrhage was approximately 3 times higher in patients who received alteplase plus heparin (1.2% vs 0.4%). Under these and other assumptions, thrombolysis resulted in marginally higher total lifetime health care costs ($43,900 vs $43,300) and was slightly less effective (10.52 vs 10.57 quality-adjusted life-years) than treatment with heparin alone. Thrombolysis was more effective and cost less than $50,000 per quality-adjusted life-year gained when we assumed that the baseline risk of death in the heparin group was 3 times the base-case value (8.1%) and that alteplase reduced the relative risk of death by at least 10%. CONCLUSIONS: Available data do not support the routine use of thrombolysis to treat patients with submassive pulmonary embolism. However, thrombolysis may prove to be cost-effective in selected subgroups of hemodynamically stable patients in whom the risk of death is higher.

Cost-Benefit Analysis↗
PubMed1984

Myocardial perforation due to temporary transvenous pacing catheters in pediatric patients.

Over a 13-year period, 18 pediatric patients received temporary transvenous pacing catheters for symptomatic bradycardia due to complete atrioventricular block or sinoatrial node dysfunction. Right ventricular wall perforation occurred in two patients. The utility of two-dimensional echocardiography in the detection and assessment of myocardial perforation by temporary pacing catheters is discussed.

Adolescent↗
PubMed1993

Myocyte DNA synthesis with aging: correlation with ventricular loading in rats.

To determine whether the detrimental mechanical and anatomical changes that occur biventricularly with aging are associated with activation of DNA synthesis, flow cytometric analysis was performed on myocyte nuclei prepared from the left and right ventricles of rats at 4, 12, 20, and 29 months of age. Heart weight increased significantly with age, and this growth adaptation was associated with the development of left ventricular failure and right ventricular dysfunction. These phenomena were coupled with marked elevations in diastolic wall stress and increases in the percentage of myocyte nuclei in S+G2M in both ventricles. Linear regression analyses revealed a direct correlation between the fraction of myocytes that entered the cell cycle and diastolic pressure and wall stress. An inverse relation was found between the percentage of myocyte nuclei in S+G2M and +dP/dt and systolic wall stress. Thus the depression of hemodynamic performance coupled with alterations in the loading conditions contributes, at least in part, to increased DNA synthesis in cardiac myocytes with age.

Aging↗
PubMedDate not supplied

Ruptured interventricular septum after blunt chest trauma: ultrasonographic diagnosis.

A 6-year-old child was found under a heavy bookcase that compressed her chest. On admission to the emergency room she was found to be dyspneic with a systolic murmur and complete atrioventricular (A-V) block. Her condition deteriorated rapidly, leading to cardiogenic shock and loss of consciousness. Echocardiographic Doppler evaluation demonstrated a large ventricular septal defect and tricuspid insufficiency. A pericardial patch was put over the tear in the septum, and torn chordae tendinae were reimplanted to the papillary muscles. A pacemaker was inserted. Her situation improved, but on the third day cardiogenic shock and right ventricular dysfunction ensued and the patient expired. A review of the previous 13 cases from the pediatric literature is presented.

Child↗
PubMed1993

Nitric oxide and prostacyclin lower suprasystemic pulmonary hypertension after cardiopulmonary bypass.

In a 3-week-old male newborn persistent suprasystemic pulmonary hypertension developed after surgical valvulotomy for a critical aortic valve stenosis. Because of a residual transvalvular pressure gradient of 35 mmHg and postoperative left as well as right ventricular dysfunction, treatment with inhaled nitric oxide (NO) and intravenously infused prostacyclin (PGI2) was attempted. Low-dose inhaled NO and low dose PGI2 corrected severe pulmonary hypertension and led to an increase in cardiac output. Treatment with NO but not PGI2 was accompanied by a rise in PaO2 and systemic blood pressure. Interruption of NO administration led to a rapid increase in pulmonary arterial pressure to suprasystemic levels. With continued i.v. PGI2 and decreasing concentrations of NO, severe pulmonary hypertension resolved after a few days suggesting that a transient endothelial dysfunction was partially responsible for pulmonary vasoconstriction. NO inhalation appears to be an effective new tool in the treatment of severe pulmonary hypertension following cardiac surgery.

Administration, Inhalation↗
PubMed1990

Aortopulmonary window coexisting with tetralogy of Fallot: echocardiographic diagnosis.

Aortopulmonary window coexisting with tetralogy of Fallot (TF) was prospectively diagnosed by two-dimensional (2D) echocardiography and Doppler in an 18-month-old boy; the diagnosis was confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed, but the patient died in the operating room from right ventricular dysfunction. The autopsy showed an adequate surgical repair, but the histologic examination of the lungs demonstrated severe pulmonary vascular disease, which was presumed to be the cause of death.

Aortopulmonary Septal Defect↗