Recombinant porcine factor VIII for high-risk surgery in paediatric congenital haemophilia A with high-titre inhibitor.

INTRODUCTION: High-titre factor VIII (FVIII) inhibitors complicate peri-operative haemostasis. Recombinant porcine FVIII (r-pFVIII) may provide an alternative haemostatic agent for high-risk procedures and allow FVIII activity monitoring. AIM: Devise an effective haemostatic plan for repair of a progressively symptomatic aortic coarctation in a 5-year-old male with immune tolerance induction (ITI) refractory high-titre FVIII inhibitors. METHODS: Preprocedure human FVIII inhibitor titre was 58 Bethesda Units mL-1 (BU) and cross-reacted to neutralize porcine FVIII at 30 BU. Daily ITI with plasma-derived FVIII concentrate was supplemented with anti-B-cell and anti-plasma cell immunotherapy to reduce FVIII inhibitor titres. Potential haemostatic agents were evaluated in comparative ex vivo thrombin generation assays (TGA). RESULTS: Four weeks after immunosuppression, human and porcine inhibitor titres declined to 16 and 2 BU respectively. TGA with r-pFVIII was less robust than with activated prothrombin complex concentrate (aPCC); however, r-pFVIII was selected for cardiac surgery to secure the ability to assay FVIII levels throughout this high-bleeding risk procedure. Haemostasis with r-pFVIII was excellent; initial trough FVIII activity levels ranged from 0.81-1.17 IU mL-1 . On postoperative day 3, peak and trough levels markedly declined suggesting a rising porcine inhibitor titre. Postprocedure prophylaxis was transitioned to aPCC, informed by TGA. CONCLUSIONS: R-pFVIII provided effective peri-procedural haemostasis with no adverse events. Rapid neutralization of r-pFVIII after the first 60 hours, despite intensive immune suppression, accentuates the importance of careful monitoring. Use of TGA can support bypassing agent selection for convalescence. The comparative cost of r-pFVIII may limit its use to high morbidity clinical scenarios.

Accuracy and pitfalls of Doppler evaluation of the pressure gradient in aortic coarctation.

Although the pressure gradient in aortic coarctation can usually be obtained by comparison of upper and lower limb blood pressures measured by sphygmomanometry, some patients may have upper or lower limb arterial compromise as a result of prior procedures or anomalous origin of the subclavian arteries, either of which may preclude accurate gradient measurement. To determine whether Doppler echocardiography could predict the pressure gradient, the Doppler method was used to predict transcoarctation gradients in 35 studies and the data were compared with the gradients measured at catheterization. Jet velocities were not adequately obtained by Doppler recording in three neonates with coarctation and patent ductus arteriosus, leaving 32 studies for analysis. The mean age of the study patients was 6 +/- 5.8 years. The mean Doppler-estimated gradient, calculated using only jet velocities distal to the obstruction (V2) in the modified Bernoulli equation, was 44 +/- 17 mm Hg, and the mean catheterization gradient was 36 +/- 21 mm Hg (p = NS; r = 0.91, SEE = 7.0 mm Hg; slope = 0.75, y = 17.3 mm Hg). The mean Doppler-estimated gradient using both the pre- and postcoarctation velocities (V1 and V2) in the modified Bernoulli equation (n = 26) was 36 +/- 20 mm Hg, and the mean catheterization gradient was 36 +/- 21 mm Hg (p = NS; r = 0.98, SEE = 4.2 mm Hg; slope = 0.91, y = 2.8 mm Hg). Doppler echocardiography closely estimated the pressure gradient in aortic coarctation, and estimation of the gradient improved when the velocities proximal as well as distal to the obstruction were included in the modified Bernoulli equation.

Measurement of cardiac output and exercise factor by pulsed Doppler echocardiography during supine bicycle ergometry in normal young adolescent boys.

The purposes of this study were to determine the ability of pulsed Doppler echocardiography to consistently and accurately measure cardiac output during exercise, and to measure the exercise factor by Doppler methodology when oxygen consumption was simultaneously measured. Thirty-four healthy young adolescent male volunteers (mean age 13 years) were recruited. Submaximal exercise was performed by supine bicycle ergometry. Cardiac output was calculated as mean velocity X cross-sectional area. Successful rest and exercise determinations of cardiac output were obtained in 81% (n = 52) of the studies. Mean cardiac output increased from 4.6 to 8.9 liters/min (p less than 0.001) during exercise and mean oxygen consumption increased from 212 to 899 ml/min (p less than 0.001). Doppler-estimated rest and exercise cardiac outputs correlated well with simultaneously measured oxygen consumption (r = 0.89, SEE = 1.2 liters/min; y = 0.006 X 3.2 liters/min). Mean exercise factor was 6.4 (1.2 SD). Twenty-six pairs of rest and exercise cardiac output determinations by Doppler technique and indirect Fick method were simultaneously compared in a subset population (r = 0.86, SEE = 1.4 liters/min; slope = 0.93, y intercept = 1.4 liters/min). Results of this study demonstrate that cardiac output and exercise factor can be estimated by pulsed Doppler echocardiography during exercise.

Doppler echocardiographic estimation of systolic pulmonary artery pressure in patients with aortic-pulmonary shunts.

The objective of this study was to determine if the pressure drop across various types of aortic-pulmonary shunts could be accurately estimated by Doppler echocardiography, and if systolic pulmonary pressure could be estimated by referencing the pressure drop across the aortic-pulmonary shunt to systolic systemic arterial pressure measured by cuff sphygmomanometry. This was done in 22 patients and Doppler results were compared with pulmonary artery pressure measured directly by strain gauge manometry. Adequate Doppler waveforms were obtained in 21 of 22 patients; 3 had a Waterston shunt, 10 had a Blalock-Taussig shunt, 1 had a left pulmonary artery-aortic anastomosis, 6 had a patent ductus arteriosus and 1 had an aortic-pulmonary window. Systolic pulmonary artery pressure estimated by Doppler echocardiography ranged from 12 to 90 mm Hg (mean 41.3 +/- 21.4 [SD] ), and measured by strain gauge manometry ranged from 20 to 90 mm Hg (mean 44.7 +/- 20.7) (p = NS, r = 0.94, SEE = 7.4 mm Hg; slope = 0.90, y intercept = 7.4 mm Hg). Systolic pulmonary artery to aortic pressure ratios predicted by Doppler recording ranged from 0.1 to 1.0 (mean 0.4 +/- 0.2 [SD] ); when calculated from direct measurement it ranged from 0.2 to 1.0 (mean 0.4 +/- 0.2) (p = NS, r = 0.92; SEE = 0.08, slope = 0.80, y intercept = 0.09). This study demonstrates that Doppler echocardiography provides an estimation of pressure drop across aortic-pulmonary shunts, and that the data can be used to estimate systolic pulmonary artery pressure by subtracting the estimated pressure drop from the systolic systemic arterial pressure.

Two-dimensional echocardiographic diagnosis of intracardiac masses in infancy.

Intracardiac masses in infancy are uncommon. Tumors, thrombi and vegetations of bacterial endocarditis are exceedingly rare in this age group. These masses are seldom diagnosed before the infant's death. In a review of two-dimensional echocardiograms performed from May 1979 to January 1981 on 741 infants less than 2 years of age, intracardiac masses were prospectively identified in 6 patients. All six patients presented with unexplained murmurs associated with either hemodynamic instability, arrhythmias or systemic emboli. One patient had a vegetation from bacterial endocarditis, one had an intracardiac thrombus associated with myocarditis, three had rhabdomyomas (two patients with tuberous sclerosis) and one had a fibro-fatty nodule attached to the eustachian valve. Two-dimensional echocardiography not only was important in the diagnosis, but also provided guidance in the medical and surgical treatment of these patients.

Doppler echocardiographic estimation of systolic pulmonary artery pressure in pediatric patients with interventricular communications.

The purpose of this study was to evaluate a noninvasive method for estimation of pulmonary artery pressures in infants and children with interventricular communications. Systolic pulmonary artery pressures measured by cardiac catheterization were compared with those estimated by Doppler echocardiography. Pressure drops were measured by Doppler study (modified Bernoulli equation) and were referenced to systolic systemic arterial pressure measured by sphygmomanometry. All 25 patients in this study had either a ventricular septal defect or a single ventricle. The systolic pulmonary artery pressure measured by cardiac catheterization ranged from 15 to 100 mm Hg (mean +/- SD 44 +/- 26) and that measured by Doppler echocardiography ranged from 5 to 100 mm Hg (mean 43 +/- 26) (p = NS; r = 0.92; SEE = 9.9; slope = 0.92; y intercept = 4.7). Systolic pulmonary artery to aortic pressure ratio measured by cardiac catheterization ranged from 0.2 to 1.0 (mean 0.5 +/- 0.3) and that measured by Doppler echocardiography ranged from 0.1 to 1.0 (mean 0.5 +/- 0.3) (p = NS; r = 0.94; SEE = 0.09; slope = 0.90; y intercept = 0.04). This study demonstrates that Doppler echocardiography can closely approximate systolic pulmonary artery pressure in patients with interventricular communications.

Delineation of site, relative size and dynamic geometry of atrial septal defects by real-time three-dimensional echocardiography.

OBJECTIVES: This study attempted to determine the site, relative size and dynamic geometry of atrial septal defects using dynamic three-dimensional echocardiography. BACKGROUND: Recent studies have demonstrated the feasibility of dynamic three-dimensional echocardiography. Images are acquired from computerized reconstruction of sequential, tomographic ultrasound "slices" of the heart. Ultrasound images can be obtained by linear progression of a transducer within a transesophageal imaging probe. In small infants and children the large transducer size has not allowed transesophageal placement, and the probe has been placed on the thorax or in the subcostal position. Other scanning devices, housed in plastic containers, acquire images in a rotational format and can also be placed in a transthoracic or subcostal position. METHODS: Specially designed transesophageal probes and a dedicated computer unit were used for two-dimensional image retrieval and reconstruction of three-dimensional images. Sixteen patients with atrial septal defects were studied (median age 18 months, range 1 day to 18 years). In one patient, images were obtained by transesophageal probe placement; in the other 15 patients, the probe was placed in the transthoracic or subcostal position. RESULTS: A dynamic three-dimensional echocardiogram of the atrial septal defect could be obtained in 13 of the 16 patients. The distinguishing features of the atrial septal defects and their spatial orientation could be visualized in unique three-dimensional views. CONCLUSIONS: Dynamic three-dimensional imaging could be applied to the specific evaluation of atrial septal defects. Unique views of the heart allowed for spatial comprehension of the defects, rendering potentially important clinical information.

Physiologic significance of chronic coronary aneurysms in patients with Kawasaki disease.

OBJECTIVES: The aim of this study was to determine whether persistent coronary aneurysms in patients with Kawasaki disease are associated with altered myocardial perfusion and function. BACKGROUND: Some patients with Kawasaki disease have died suddenly because of severe coronary artery stenosis; others have chronic coronary aneurysms. METHODS: Eleven patients with chronic coronary aneurysms were enrolled in the study. The size of the aneurysms and the degree of associated stenosis were determined by angiography in nine patients and by echocardiography in two. All patients underwent simultaneous function and myocardial perfusion assessment during symptom-limited exercise by echocardiography and technetium-99m sestamibi imaging, respectively. RESULTS: Of 33 vascular territories, 18 contained coronary aneurysms measuring 3.5 to 10 mm. Three aneurysms were associated with significant stenosis as detected by angiography. Of the 18 vascular territories, 13 were normal, and 5 manifested stress-induced perfusion defects; of the latter 5 areas, 4 had associated wall motion abnormalities. The three territories supplied by stenotic coronary arteries had both abnormal regional function and perfusion demonstrated during exercise. CONCLUSIONS: Patients with chronic coronary aneurysms may have associated stenosis, as detected by angiography, with a subjacent myocardium that is subject to abnormal perfusion and function. However, the majority of coronary aneurysms are associated with normal regional coronary flow reserve, as assessed by myocardial perfusion imaging, and even giant coronary aneurysms may be associated with normal coronary flow reserve and preserved regional myocardial function during stress.

Noninvasive assessment of hemodynamic responses to exercise in pulmonary regurgitation after operations to correct pulmonary outflow obstruction.

The influence of pulmonary regurgitation (PR) on exercise capacity is unknown. The hemodynamic responses to exercise in postoperative patients with PR was determined using Doppler-measured regurgitant fraction to indicate PR severity. Maximal heart rate, oxygen consumption and workload capacity were measured during upright cycle ergometry. Cardiac output was measured at rest and during submaximal supine cycle ergometry by pulsed Doppler echocardiography. Oxygen consumption was simultaneously measured and exercise factor was calculated as the change in cardiac output per change in oxygen consumption. Twenty-seven patients were compared with 17 age-, size- and sex-matched control subjects. Patients with PR had larger right ventricles (p less than or equal to 0.001), lower heart rate response (p less than or equal to 0.05), lower maximal oxygen consumption (p less than or equal to 0.005) and lower workloads (p less than or equal to 0.005) when compared with normal control subjects during maximal exercise testing. Exercise factor was the same for both groups. Patients with PR were then separated into mild, moderate and severe groups. Patients with mild PR had a normal response to exercise. However, patients with moderate and severe PR had lower maximal oxygen consumptions and maximal workloads than control subjects. Control, mild and moderate PR groups had similar exercise factors. Patients with severe PR had markedly low cardiac output responses. PR is associated with reduced exercise capability, which is related to the severity of the PR.

Relation of Still's murmur, small aortic diameter and high aortic velocity.

The origin of Still's innocent murmur, first described in 1909, is obscure. Seventy normal children and young adults, 29 with Still's murmur and 41 with no murmur, were studied. Pulsed Doppler and 2-dimensional echocardiography were used to evaluate possible causes, including tricuspid regurgitation, left ventricular bands, ascending and descending aortic and pulmonary velocities, ascending aortic diameter, and magnitude of spectral widths. Mean ascending aortic diameter relative to body surface area was significantly smaller for the group with Still's murmur (p less than 0.001). Since cardiac output was similar for the 2 groups, the average peak ascending velocity (133 cm/s) and average peak descending aortic velocity (118 cm/s) were significantly higher in the innocent murmur group as compared to similar respective means in the control group without the murmur (107 and 104 cm/s, respectively) (p less than 0.001 and p less than 0.01, respectively). No significant differences were found when the 2 groups were compared with respect to mean peak pulmonary artery velocity adjusted for body size, spectral widths in the ascending and descending aorta and in the pulmonary artery, and the presence of tricuspid regurgitation or ventricular bands. These observations suggest that the origin of Still's murmur is related to a small ascending aortic diameter with concomitant high aortic blood flow velocity.

Transatrial septal velocity measurement by Doppler echocardiography in atrial septal defect: correlation with Qp:Qs ratio.

Right atrial velocities measured perpendicular to the atrial septum by Doppler echocardiography in patients with atrial septal defects (ASD) have a discernible morphology that may bear a relation to shunt magnitude. The integral of the right atrial Doppler waveform was compared with shunt magnitude measured at cardiac catheterization or nuclear shunt scan in 17 ASD patients. For control subjects, the mean right atrial velocity was 15 +/- 4 cm/s (+/- standard deviation) and that for ASD patients was 41 +/- 11 cm/s (p less than 0.001). Doppler pulmonary-to-systemic flow ratio (Qp:Qs) correlated with catheterization Qp:Qs ratio (n = 9, r = 0.85, SEE = 0.27) and with nuclear Qp:Qs ratios (n = 8, r = 0.60, SEE = 0.51). Mean transatrial septal velocity in ASD patients correlated with catheterization Qp:Qs ratio (n = 9, r = 0.8, SEE = 6.0) and with simultaneous Doppler Qp:Qs ratio (n = 16, r = 0.89, SEE = 4.9, y = 16.2 +/- 8.3). Although Qp:Qs ratio can be approximated by measuring pulmonary and systemic flow by Doppler echocardiography in many ASD patients, this newly described method allows estimation of Qp:Qs ratio. It is useful when these more conventional measurements cannot be performed because of turbulence or when inadequate imaging prevents Doppler pulmonary to systemic flow measurement.

Balloon dilation angioplasty for coarctation of the aorta.

Eleven patients with coarctation of the aorta (C of A) underwent balloon dilation angioplasty at the University of Arizona from November 1983 to January 1985. Eight had previously undergone surgery and 3 had native C of A. Two operations were considered unsuccessful: 1 in a patient who underwent tube graft--descending aortic anastomosis narrowing and 1 in a patient with a native wedge type of C of A. Overall mean gradient fell from 47 to 13 mm Hg immediately after the procedure. Mean gradient at repeat catheterization in 7 patients (mean 8 months after angioplasty) was 6 mm Hg. Five patients showed a transient increase in the gradient measured on the day after angioplasty, with 3 showing a fairly marked increase. Values returned to levels equal to or less than gradients measured immediately after the procedure. Angiographic findings at follow-up catheterization in 7 patients showed no evidence of aneurysm formation in either the operative group or in the 2 patients with native C of A who had a membrane type of deformity. Mean C of A to ascending aortic diameter ratios increased from 0.44 to 0.80. At repeat angiography, the mean ratio was 0.76 in the 7 patients studied. Further longitudinal studies in these patients are necessary before reaching conclusions about the advantage of this procedure over surgery, but these early longitudinal results are encouraging for the populations studied: postoperative patients and patients with native membrane types of C of A.

Fate of mitral regurgitation following repair of atrioventricular septal defect.

The purpose of this study was to evaluate the fate of mitral regurgitation (MR) following repair of atrioventricular septal defects (AVSDs). Echocardiograms of all survivors of isolated AVSD surgery between 1986 and 1996, who had had > or =2 postoperative color Doppler studies (39 patients), were reviewed. On each study, MR severity was graded on a 1+ to 4+ scale, based upon the size of the MR jet. Median age at surgery was 9 months (range 3 to 169); median age at postoperative follow-up was 45 months (range 3 to 107). Mild deterioration of mitral valve function was fairly common. MR severity increased by > or =1 grade in 16 patients (41%) during the course of the study. However, the deterioration in mitral valve function occurred primarily during the early postoperative time intervals. After the initial 32 postoperative months, MR worsened on only 4 occasions and in each instance worsened by only 1 grade. Deterioration to 4+ MR occurred in only 3 patients, and was not observed after the initial 30 postoperative months. Survival curve analysis predicted a 90% probability of not having severe (4+) MR after 30 months (lower 95% confidence bound: 80%). Postoperative MR remains fairly stable following AVSD repair. Serious deterioration is rare, especially after the initial 30 postoperative months.

Echocardiographic characteristics of successful deployment of the Das AngelWings atrial septal defect closure device: initial multicenter experience in the United States.

The AngelWings device is a newer transcatheter device used for closure of secundum atrial septal defects (ASD) and patent foramen ovale (PFO), which consists of a self-centering, 2-disk system. Transesophageal echocardiography (TEE) plays a pivotal role in the deployment of the 2 disks of this device, on the appropriate sides of the atrial septum. The objective of this study is to describe the echocardiographic findings associated with successful deployment of the AngelWings device for closure of ASD and PFO. We evaluated the TEE studies of 70 patients enrolled in 4 United States centers, for closure of ASD and PFO with the AngelWings device. The TEE characteristics of successful and unsuccessful deployments were analyzed. Residual shunts across the atrial septum were assessed by TEE at the end of the procedure, 24 hours later by transthoracic echocardiography, and at 6 months by TEE. The deployment of the device was successful in 65 patients (93%). In the unsuccessful group, ASD size by TEE was larger (13.4 +/- 3.1 vs 8.9 +/- 4.7 mm, p <0.05). TEE was successful in identifying snagging of the device by intracardiac structures and prolapse of corners of the left or right atrial disk through the ASD, features that were difficult to identify by fluoroscopy. The echocardiographic characteristics outlined here are important guidelines for successful deployment of the AngelWings device.

Effect of pulmonary artery stenoses on the cardiopulmonary response to exercise following repair of tetralogy of Fallot.

Data from exercise tests, echocardiograms, and lung perfusion scans were analyzed to determine whether the excessive minute ventilation (VE) often encountered among patients with tetralogy of Fallot is due to ventilation-perfusion mismatch secondary to branch pulmonary artery stenoses. Patients with branch PA stenoses had lower peak oxygen consumptions and higher VE during exercise than did patients without stenoses, and a strong correlation existed between the degree of pulmonary blood flow maldistribution on lung perfusion scan and the amount of excessive VE during exercise.

Possible new autosomal recessive syndrome of lymphedema, hydroceles, atrial septal defect, and characteristic facial changes.

We describe two brothers with congenital lymphedema of lower limbs, atrial septal defect (ASD), and similar facial appearance. A sister had severe hydrops fetalis, ASD, omphalocele, and other anomalies. This combination of congenital lymphedema and ASD differs from other reported cases of congenital lymphedema and most likely constitutes a previously unrecognized autosomal recessive syndrome.

Valve-sparing operation for balloon-induced aortic regurgitation in congenital aortic stenosis.

OBJECTIVE: Aortic regurgitation after balloon dilation of congenital aortic stenosis may be treated with valve repair as an alternative to replacement. METHODS: Charts and echocardiograms of all patients undergoing aortic valve operations after balloon dilation of congenital aortic stenosis at our institution between January 1988 and December 1999 were reviewed. RESULTS: Twenty-one patients underwent valvuloplasty for predominant aortic regurgitation 9 months to 15 years (mean, 6.1 years) after balloon dilation. The mean +/- SD age at the time of the operation was 11 +/- 7 years. Aortic regurgitation was caused by a combination of commissural avulsion (10), cusp dehiscence with retraction (9), cusp tear (5), central incompetence (2), perforated cusp (1), or cusp adhesion to the aortic wall (1). Repair techniques included commissural reconstruction with a pericardial patch (8), pericardial patch cusp augmentation (6), primary suture repair (6), raphae release and debridement (4), commissurotomy (4), commissural resuspension with sutures (3), and cusp release (1). There were no deaths. At a mean follow-up of 30.1 months (range, 9 months-8 years), all patients were asymptomatic, and the grade of aortic regurgitation had been significantly reduced (P <.001). Left ventricular end-diastolic dimension z scores and proximal regurgitant jet/aortic anulus diameter ratios were significantly reduced (P <.001) and remained so over time. Freedom from reoperation for late failure was 100%, and overall freedom from reintervention was 80% at 3 years. CONCLUSION: Aortic valve repair for balloon-induced aortic regurgitation is reproducible and durable at medium-term follow-up.

Prenatal diagnosis of congestive heart failure in a fetus with a sacrococcygeal teratoma.

Two-dimensional Doppler echocardiography was used to diagnose congestive heart failure in a fetus with a large sacrococcygeal teratoma. Ultrasound performed for size-date inconsistency revealed a 27.5-week fetus with hydrops and a large solid and cystic mass in the sacral region. Fetal echocardiography showed dilated ventricles and a pericardial effusion; Doppler ultrasound demonstrated increased velocities and volume flows, along with tricuspid and mitral regurgitation. At delivery, the mass was bleeding actively, the amniotic fluid was markedly bloody, and the neonatal hematocrit was 10%. We postulate that intrauterine hemorrhage from the teratoma led to anemia and high-output cardiac failure confirmed by Doppler echocardiography, and suggest that all fetuses with sacrococcygeal teratomas be evaluated by two-dimensional Doppler echocardiography to detect the presence of congestive heart failure, in order to allow well-timed therapeutic interventions.

Cardiac Doppler flows during fetal arrhythmias: physiologic consequences.

Eighty-six fetuses of 21-41 weeks' gestation with arrhythmias were studied with ultrasound and heart rate monitoring. The type of arrhythmia was identified by M-mode studies and was confirmed by postnatal electrocardiogram in 70 infants. The most common arrhythmia was premature atrial contractions (76), followed by premature ventricular contractions (five), paroxysmal supraventricular tachycardia (four), and atrial fibrillation/flutter (one). Doppler echocardiography was performed in 54 fetuses to measure flow velocities across the atrioventricular and semilunar valves. After isolated premature atrial and ventricular contractions, post-extrasystolic potentiation was demonstrated by an increase in fractional shortening (N = 32) of 49 +/- 6% in the right ventricle and 64 +/- 7% in the left ventricle. When post-extrasystolic beats were compared with normal beats, Doppler-determined time-velocity integrals increased 43% across the tricuspid valve, 41% across the mitral valve, 34% across the pulmonary valve, and 38% across the aortic valve. Mean velocity increased significantly after conversion to normal sinus rhythm in the five fetuses with supraventricular tachycardia (P less than .05). By studying the physiologic consequences of fetal arrhythmias using two-dimensional Doppler and M-mode ultrasound, we have documented the presence of post-extrasystolic potentiation after premature contractions, the existence of the Frank-Starling mechanism, and an increase in mean velocity (and therefore in cardiac output) after conversion of fetal tachyarrhythmias to normal sinus rhythm.

Cardiac dysfunction in twin-twin transfusion syndrome: a prospective, longitudinal study.

OBJECTIVE: To use serial echocardiography to evaluate prospectively the cardiac dysfunction in twin-twin transfusion syndrome and determine its clinical course and outcome. METHODS: Twin pregnancies presenting in the second trimester with sonographic evidence of twin-twin transfusion syndrome were managed with therapeutic reduction amniocenteses. Gestational age at diagnosis and delivery, number of amniocenteses performed, volume of amniotic fluid withdrawn, placentation, birth weight, hemoglobin at delivery, and perinatal outcome were recorded. Serial fetal echocardiography was carried out in a single tertiary center. Echocardiographic assessments included cardiac anatomy, chamber size, cardiothoracic ratio, interventricular septal thickness, ventricular systolic function, and the presence and severity of atrioventricular valve regurgitation. Postnatal echocardiograms were obtained on the surviving twins. RESULTS: Twelve cases of twin-twin transfusion syndrome were evaluated with serial echocardiography. Evidence of cardiac dysfunction was present prenatally in 10 recipient twins. All of the donor twins had normal fetal echocardiographic assessments. The most common abnormalities detected prenatally in recipient twins were decreased ventricular function, tricuspid regurgitation, and cardiac chamber enlargement. A deterioration of cardiac function was observed in seven recipient twins with increasing gestational age. Four of the eight surviving recipient twins had persistent postnatal echocardiographic abnormalities on follow-up examinations after the first 28 days of life. CONCLUSION: Prenatal cardiac dysfunction is common in recipient twins and can be transient, progressive, or persistent beyond the neonatal period.