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Biomedical subjects

J Rychik

Publications and source records attributed to J Rychik.

At least 55 records · Page 3Linked to original sources
PubMed1996

Quantitative assessment of myocardial tissue velocities in normal children with Doppler tissue imaging.

Assessment of myocardial tissue velocities in children in feasible with DTI. We have described normal velocity values for left ventricular motion in children during systole and diastole, at the mitral annulus, and at the posterior wall. The pattern of mitral annular motion in diastole mimics that of mitral blood inflow, whereas the pattern of posterior wall motion differs and may provide for a new form of assessment of diastolic function. Further investigation of myocardial velocities in states of volume and pressure overload, as seen in various forms of congenital heart disease, is warranted.

Adolescent↗
PubMed1996

Protein-losing enteropathy after Fontan operation: resolution after baffle fenestration.

A 4-year-old child with hypoplastic left heart syndrome and a 6-year-old child with tricuspid atresia had both undergone staged reconstructive operations culminating in a Fontan operation. Peripheral edema, ascites, and hypoalbuminemia refractory to dietary manipulation and steroid therapy developed in both patients. After hemodynamic assessment, each child underwent surgical creation of a 4.8-mm fenestration in the previously placed baffle that separated the systemic venous pathway from the pulmonary venous atrium. Peripheral edema and ascites promptly resolved and serum protein levels normalized within 2 weeks after operation. Systemic arterial saturation is 86% in each child, and both children remain clinically well with no evidence of protein-losing enteropathy on normal diets and without specific medical therapy.

Ascites↗
PubMed1996

Morphometric analysis of unbalanced common atrioventricular canal using two-dimensional echocardiography.

OBJECTIVES: This study was designed to define morphometric echocardiographic variables of unbalanced common atrioventricular canal (CAVC) that could aid in appropriate referral for surgical repair. BACKGROUND: Unbalanced CAVC has a high surgical mortality rate. This may be secondary to inappropriate referral of some patients for two-ventricle repair (closure of septal defects) instead of single-ventricle repair (Norwood palliation and Fontan operation). METHODS: The echocardiograms of 103 patients with CAVC were retrospectively reviewed. In the subcostal left anterior oblique view, the area of the atrioventricular (AV) valve aportioned over each ventricle was measured, and an AV valve index (AVVI) was calculated as left/right valve area. The ventricular cavity ratio between the two ventricles was estimated as left ventricular length times width divided by right ventricular length times width. These variables were correlated with surgical referral and outcome. RESULTS: Patients previously categorized as having balanced CAVC all had AVVI > 0.67 (n = 77). Of the patients with unbalanced CAVC (n = 26), 11 had ductal-dependent circulation and underwent Norwood palliation (AVVI 0.21 +/- 0.13, mean +/- SD), and 15 had two-ventricle repair (AVVI 0.51 +/- 0.12, p < 0.0001). Of these 15 patients, 9 have survived, with no difference in mean AVVI between survivors and nonsurvivors (0.52 +/- 0.11 versus 0.49 +/- 0.13, p = 0.72). For all 103 patients, AVVI correlated with ventricular cavity ratio. However, of the unbalanced CAVC group who underwent two-ventricle repair, three nonsurvivors had a discrepancy between AVVI and ventricular cavity ratio (low AVVI but normal ventricular size). A large ventricular septal defect was present in all six nonsurvivors but in only four of nine survivors (p < 0.05). CONCLUSIONS: Echocardiographic morphometry is useful in defining unbalance in CAVC. If AVVI is < 0.67 in the presence of a large ventricular septal defect, a single-ventricle approach to repair should be considered.

Child, Preschool↗
PubMed1996

Early reduction of the volume work of the single ventricle: the hemi-Fontan operation.

BACKGROUND: In hearts with a functional single ventricle, cavity volume and myocardial muscle mass increase as a consequence of the excessive volume work associated with parallel pulmonary and systemic circulations. The hemi-Fontan operation was conceived as a means of accomplishing early reduction of the volume work of the single ventricle. METHODS: All patients presenting in infancy with single-ventricle physiology were managed by early hemi-Fontan operation in anticipation of a subsequent completion Fontan operation. Between May 1989 and August 1995, 400 patients less than 2 years of age underwent hemi-Fontan operations. Mean age at operation was 8.5 months (range, 2 months to 24 months). The hemi-Fontan operation included association of superior vena(e) cava(e) with the branch pulmonary arteries, augmentation of the central pulmonary arteries, occlusion of the inflow of the superior vena cava into the right atrium, and elimination of other sources of pulmonary blood flow. RESULTS: Operative mortality ( < 30 days) was 31 of 400 patients (7.8%). For the last 200 patients, operative mortality was 8 of 200 (4.0%). Younger age at operation was not an independent risk factor for operative mortality. Urgent operation in the presence of a hemodynamic burden requiring concomitant procedures was associated with increased mortality. CONCLUSIONS: The hemi-Fontan operation can be accomplished with low operative mortality in young patients, achieving early reduction of the volume work of the single-ventricle heart.

Age Factors↗
PubMed1995

Interrupted aortic arch. Impact of subaortic stenosis on management and outcome.

Interrupted aortic arch (IAA) is often related developmentally to subaortic obstruction (SAO). When severe, SAO must be addressed in surgical management of IAA. From 1990 to 1993, 25 neonates presented for initial surgical management of IAA complexes. Associated lesions were ventricular septal defect (VSD) with or without atrial septal defect (19 patients), truncus arteriosus (3 patients), tricuspid atresia with transposition of the great arteries (1 patient), aortic atresia with VSD (1 patient), and d-transposition of the great arteries with VSD (1 patient). Overall hospital mortality was 20% (five deaths). One death was related to sepsis and two to sudden hemodynamic decompensation (a 2-kg premature infant after arch repair and VSD closure and a neonate with IAA-truncus arteriosus after arch repair and truncus repair with aortic root replacement). Two deaths were related to low cardiac output in patients with severe subaortic narrowing (< 3 mm by two-dimensional echocardiography), which was not addressed surgically. Of 10 additional patients judged preoperatively to have severe SAO, 1 underwent resection of the infundibular septum together with VSD closure and arch reconstruction, and 9 underwent a modification of Norwood's operation with arch reconstruction and proximal pulmonary artery to aortic anastomosis (7 with systemic to pulmonary artery shunts and 2 with right ventricle to pulmonary artery outflow tract reconstruction). One patient died 2 months after surgery of staphylococcal sepsis. All 9 others were discharged well. Subaortic narrowing is a physiologically important element of IAA complexes. When SAO is severe, satisfactory initial palliation can be achieved by a modification of Norwood's operation.

Aorta, Thoracic↗
PubMed1995

Avoidance of subaortic obstruction in staged management of single ventricle.

BACKGROUND: Subaortic obstruction is a frequent accompaniment of single-ventricle anatomy. Most often, the aorta arises from an outflow chamber that is connected to the single ventricle by a bulboventricular foramen or ventricular septal defect. This connection may be restrictive of flow at birth, or may become obstructive after surgical procedures that reduce the volume work of the ventricle. Subaortic obstruction is recognized as a risk factor for reconstructive surgical procedures for single ventricle. METHODS: To prevent the consequences of subaortic obstruction, we have routinely amalgamated the proximal main pulmonary artery with the ascending aorta and arch early in the management of these patients. From September 1990 through September 1994, 29 neonates and infants with single ventricle and established or potential subaortic obstruction underwent staged reconstructive surgical procedures. The initial operation in the newborn period was a Norwood procedure (18 patients) or a pulmonary artery band (5 patients). All survivors underwent a hemi-Fontan procedure at approximately 6 months. RESULTS: Eighteen patients have undergone a completion Fontan operation with no deaths. Five await completion Fontan. None has subaortic obstruction, and none has pulmonary valve insufficiency that is graded more than mild. CONCLUSIONS: Early association of the proximal main pulmonary artery with the ascending aorta appears to obviate the risks and complications associated with subaortic obstruction in patients with single ventricle.

Cardiac Surgical Procedures↗
PubMed1995

Acute changes in left ventricular geometry after volume reduction operation.

BACKGROUND: After surgical removal of a volume load, regression of myocardial mass proceeds slowly relative to diminution in ventricular cavity size, resulting in increased wall thickness and decreased cavity dimensions, which may affect the filling properties and performance of the heart. We investigated the acute changes in ventricular geometry that occur after the Fontan operation and hemi-Fontan operation for tricuspid atresia, and compared them with closure of a ventricular septal defect in a two-ventricle heart. METHODS: We reviewed the results of echocardiography performed before and 8 +/- 7 days after (1) Fontan operation for tricuspid atresia (n = 9), (2) hemi-Fontan operation for tricuspid atresia (n = 10), and (3) closure of a ventricular septal defect (n = 13). Measurements were made from images of the left ventricle at end-diastole: (1) apical, septal, and posterior wall thickness; and (2) long- and short-axis cavity diameters, cross-sectional areas, and ventricular volume. Posterior wall thickness to cavity dimension ratio was calculated. RESULTS: Wall thickness increased in all groups, with the greatest degree of increase after the Fontan operation. Cavity measures decreased most dramatically after the Fontan operation, with less dramatic and equivalent changes noted after the hemi-Fontan operation and ventricular septal defect closure. Posterior wall thickness to cavity diameter ratios were equivalent in all before operation, increased after operation, and were greatest after the Fontan operation. CONCLUSIONS: Changes in ventricular geometry identified as an increase in wall thickness and a decrease in cavity dimension are most dramatic after the Fontan operation. Changes seen after the hemi-Fontan operation are of a milder degree, which may in part explain the excellent clinical course after this operation.

Child, Preschool↗
PubMed1995

Early changes in ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery.

OBJECTIVES: This study investigated the phenomenon of, and the relation between, alterations in ventricular geometry after acute surgical volume unloading of the ventricle and the development of subaortic stenosis in patients with a single ventricle and ventricular septal defect-dependent systemic flow. BACKGROUND: Subaortic outflow obstruction has been observed to occur in patients with a single left ventricle after placement of a pulmonary artery band. The timing and etiology of this phenomenon are not well defined. METHODS: The preoperative and postoperative echocardiograms of 18 patients 14.9 +/- 22.8 months old (mean +/- SD) with a diagnosis of single left ventricle who underwent pulmonary artery banding or cavopulmonary connection were reviewed. Postoperative studies were performed a mean of 7.0 +/- 6.5 days after operation. The ventricular septal defect diameter was measured in two orthogonal views and the area calculated using the formula for an ellipse. Interventricular septal and posterior wall thickness and left ventricular diameter and length were also measured. RESULTS: Mean ventricular septal defect area indexed to body surface area diminished by 36 +/- 23% (3.1 +/- 2.7 to 2.0 +/- 1.8 cm2/m2, p < 0.01). Mean interventricular septal and posterior wall thickness increased significantly, and left ventricular diameter and length decreased significantly. A greater diminution in ventricular septal defect area was noted after cavopulmonary connection (41 +/- 19%, p < 0.01) than after pulmonary artery banding (25 +/- 28%, p = 0.22). CONCLUSIONS: In the single left ventricle, diminution in ventricular septal defect size occurs early and is related to an acute alteration in ventricular geometry that accompanies the decrease in ventricular volume. Ventricular septal defect diminution was greater after volume unloading of the ventricle after cavopulmonary connection than after pulmonary artery banding.

Aortic Stenosis, Subvalvular↗
PubMed1995

Results of Norwood's operation for lesions other than hypoplastic left heart syndrome.

Norwood's operation provides satisfactory palliation for neonates with hypoplastic left heart syndrome. The dominant physiologic features of hypoplastic left heart syndrome, ductal dependency of the systemic circulation and parallel pulmonary and systemic circulations, are shared by a multitude of other less common congenital heart malformations. Theoretically, these should be equally amenable to palliation by Norwood's operation. Between January 1990 and June 1994, 60 neonates with malformations other than hypoplastic left heart syndrome underwent initial surgical palliation by Norwood's procedure. Diagnoses included single left ventricle with levo-transposition of the great arteries (12); critical aortic stenosis (8); complex double-outlet right ventricle (8); interrupted aortic arch with ventricular septal defect and subaortic stenosis (7); ventricular septal defect, subaortic stenosis, and coarctation of the aorta (7); aortic atresia with large ventricular septal defect (6); tricuspid atresia with transposition of the great arteries (6); heterotaxy syndrome with subaortic obstruction (3); and other (3). There were 10 hospital deaths and 50 survivors (83% survival). After the introduction of inspired carbon dioxide therapy into the postoperative management protocol (1991), 42 of 47 patients survived (89% survival). Mortality was independent of diagnosis and essentially the same as that for hypoplastic left heart syndrome. With minor technical modifications, Norwood's operation provides satisfactory initial palliation for a wide variety of malformations characterized by ductal dependency of the systemic circulation in anticipation of either a Fontan procedure or a biventricular repair.

Aorta, Thoracic↗
PubMed1994

Early changes in ventricular geometry and ventricular septal defect size following Rastelli operation or intraventricular baffle repair for conotruncal anomaly. A cause for development of subaortic stenosis.

BACKGROUND: Obstruction to left ventricular outflow can be seen after surgical repair of congenital heart disease in which a ventricular septal defect (VSD) is closed by means of a baffle to the systemic great artery arising from the right ventricle (Rastelli operation, intraventricular repair, for conotruncal anomaly). We investigated the hypothesis that obligatory volumetric changes that occur after this operation lead to acute alterations in ventricular geometry and VSD size, resulting in subsequent subaortic stenosis in patients who were thought before operation to have a large, nonrestrictive VSD orifice. METHODS AND RESULTS: Preoperative and postoperative echocardiograms and medical records of 24 patients with conotruncal anomaly who underwent conventional Rastelli operation or intraventricular repair in which the VSD was used as part of a new left ventricular outflow were reviewed. Eleven patients had transposition of the great arteries with pulmonic stenosis, 7 had double-outlet right ventricle, and 6 had subaortic atresia or stenosis with a normal-size left ventricle and underwent Norwood's palliation in infancy. All had large, nonrestrictive VSDs at preoperative cardiac catheterization. The mean age at the time of surgery was 32 +/- 24 months. The following measurements were made from two-dimensional echocardiographic images obtained before and 5 +/- 4 days after surgery from the subcostal views at end diastole: (1) VSD diameter; (2) short-axis left ventricular internal diameter (LVID); (3) left ventricular posterior wall thickness (LVPW); and (4) systemic great artery diameter (arising from the right ventricle). VSD diameter diminished significantly after surgery (11.6 +/- 3.6 versus 10.1 +/- 3.7 mm, P < .0001), as did LVID (34.9 +/- 5.0 versus 31.7 +/- 5.1 mm, P < .001). LVPW thickness increased significantly (5.7 +/- 1.0 versus 6.7 +/- 1.1 mm, P < .0001), while great artery diameter was unchanged (16.2 +/- 4.0 versus 16.7 +/- 3.8 mm, P = NS). Percent change in VSD dimension correlated with percent change in LVPW/LVIDD ratio (degree of ventricular "contraction"). Nine patients subsequently developed subaortic obstruction at the VSD orifice level and had a greater degree of early diminution in VSD size (21 +/- 8% versus 10 +/- 8%, P < .002) as well as postoperative change in LVPW/LVID ratio (0.24 +/- 0.04 versus 0.20 +/- 0.02, P < .002) than those who did not develop subsequent subaortic obstruction. CONCLUSIONS: The left ventricle undergoes geometric change after Rastelli operation or intraventricular repair, surgeries in which the VSD is used as the new left ventricular outflow. These changes are manifested as increased wall thickness, decreased cavity dimensions, and a decrease in VSD size. Patients who subsequently develop left ventricular outflow obstruction have the greatest degree of ventricular contraction and VSD diminution early after surgery.

Child, Preschool↗
PubMed1991

Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis.

Between December 1985 and April 1990, 50 infants with a variety of congenital cardiac lesions other than the hypoplastic left heart syndrome underwent surgical relief of aortic outflow obstruction by creation of a pulmonary artery to aorta anastomosis. The patients were grouped anatomically by ventriculoarterial alignment. Nineteen had normally aligned great arteries (group I); 25 had transposition of the great arteries, all with a univentricular heart of left ventricular morphology (group II); and 6 had a double-outlet right ventricle (group III). All patients had either aortic stenosis with atresia, subaortic stenosis or a restrictive ventricular septal defect. Sixteen had normal arch anatomy; 34 had arch anomalies consisting of arch hypoplasia (n = 17), coarctation (n = 11), interruption of the arch (n = 4) and complex arch anomalies (n = 2). Surgery was performed at a median age of 10 days (range 2 to 184). Of the 50 infants, 33 survived. No significant difference in early survival (30 days) was noted among the groups of varying ventriculoarterial alignment (68% group I, 72% group II, 83% group III) (p greater than 0.05). Overall actuarial survival was 63% at 18 months. Analysis of actuarial survival by arch anatomy, although not statistically significant, revealed a trend toward better survival at 18 months postoperatively in infants with normal arch anatomy (81%) than in infants with arch anomalies (54%). Of the 33 survivors, 26 have proceeded to the next surgical stage, including the Fontan procedure in 8, superior cavopulmonary anastomosis in 13 and biventricular repair in 5.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗
PubMed1991

Doppler color flow mapping assessment of residual shunt after closure of large ventricular septal defects.

Of 179 consecutive patients who underwent closure of a large ventricular septal defect (VSD) as part of reparative surgery under 2 years of age, 19 died early and 160 had echocardiographic evaluation that included Doppler color flow mapping at 8 +/- 7 days after repair. Sixty-one (38%) of patients had evidence of residual shunting in the peripatch area. Two independent observers measured the Doppler color jet width at the left septal surface; jet width was correlated with subsequent reoperation for residual VSD. Patients were divided into two groups by jet width measurement: group 1 measured 4 mm or greater (n = 4), and group 2 measured less than 4 mm (n = 57). Of the group 1 patients, three returned to the operating room for a large residual defect; one patient died without reoperation but was confirmed to have had a large defect by angiography. Four patients in group 2 underwent surgery for a residual VSD; two underwent reoperation for reasons other than a residual VSD and had closure of a small residual VSD; one patient thought to have a large residual VSD at reoperation was noted to have multiple muscular VSD and only a small residual VSD; and one patient had a small defect initially but returned 2 years later with late patch dehiscence. Sixty-five percent of 23 group 2 patients reevaluated at a mean of 10 +/- 8 months after initial study no longer showed evidence of residual shunting. Residual Doppler color jets are frequently noted after repair of a large VSD.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Catheterization↗
PubMedDate not supplied

Fetal cardiovascular physiology.

The cardiovascular system of the fetus is physiologically different than the adult, mature system. Unique characteristics of the myocardium and specific channels of blood flow differentitate the physiology of the fetus from the newborn. Conditions of increased preload and afterload in the fetus, such as sacrococcygeal teratoma and twin-twin transfusion syndrome, result in unique and complex pathophysiological states. Echocardiography has improved our understanding of human fetal cadiovasvular physiology in the normal and diseased states, and has expanded our capability to more effectively treat these disease processes.

Adult↗
PubMedDate not supplied

Transcatheter radiofrequency ablation for congenital junctional ectopic tachycardia in infancy.

Congenital junctional ectopic tachycardia (JET) is a difficult to treat arrhythmia with a variably poor response to pharmacologic intervention. We report on the successful treatment of a 17-day-old infant with JET via transcatheter radiofrequency ablation of the arrhythmogenic focus resulting in resolution of the tachycardia and maintenance of normal atrioventricular nodal function. Transcatheter radiofrequency ablation techniques should be considered in infants with life-threatening arrhythmia recalcitrant to standard forms of drug therapy.

Catheter Ablation↗
PubMedDate not supplied

Right ventricular function in congenital heart disease: pressure and volume overload lesions.

The right ventricle is often subject to both pressure and volume overload in congenital heart disease. Evaluating right ventricular function in both the native lesion and after surgery in light of these loading conditions, presents a unique challenge for investigators studying these misshapen hearts. The purpose of this article is to briefly delineate what is generally known about right ventricular function in congenital heart disease and to touch on some noninvasive imaging modalities which have helped shed some light on this matter.

Animals↗
PubMedDate not supplied

Doppler echocardiographic analysis of flow in the ductus arteriosus of infants with hypoplastic left heart syndrome: relationship of flow patterns to systemic oxygenation and size of interatrial communication.

Excessive pulmonary overcirculation related to imbalance in the pulmonary/systemic vascular resistance ratio contributes to hemodynamic instability in infants with the hypoplastic left heart syndrome. Because the ductus arteriosus bridges the two vascular circuits in this lesion, we studied the Doppler echocardiographic flow patterns in the ductus arteriosus of 50 infants with hypoplastic left heart syndrome to investigate their relationship to the degree of pulmonary blood flow as measured by simultaneously obtained levels of partial pressure of oxygen in arterial blood. The degree of restriction to pulmonary venous egress as determined by size of the interatrial communication was also correlated with ductal flow patterns and partial pressure of oxygen in arterial blood. Biphasic flow was noted in all infants. Mean peak velocity of antegrade flow (pulmonary artery to aorta) was greater than that of retrograde flow (aorta to pulmonary artery) (131 +/- 45 cm/sec versus 54 +/- 15 cm/sec; p < 0.001), mean time of retrograde flow was greater than that of antegrade flow (246 +/- 60 msec versus 174 +/- 28 msec; p < 0.001), and mean velocity-time integral of antegrade flow was greater than that of retrograde flow (13.3 +/- 4.8 cm versus 6.3 +/- 3.4 cm; p < 0.001). The ratio of velocity-time integral of retrograde flow/antegrade flow (a volumetric estimate of diastolic reversal into the pulmonary vascular bed indexed to systemic output) correlated extremely well with partial pressure of oxygen in arterial blood (r = 0.91; p < 0.0001). Categoric size of the interatrial communication (none, n = 2; small [<2 mm], n = 9; moderate [3 to 4 mm], n = 23; and large [>4 mm], n = 16) correlated with partial pressure of oxygen in arterial blood (r = 0.82; p < 0.001); the smaller the interatrial communication the lower the partial pressure of oxygen in arterial blood and velocity-time integral ratio of retrograde/antegrade flow. Doppler flow patterns in the ductus arteriosus of infants with hypoplastic left heart syndrome are reflective of the resistance ratio between the pulmonary and systemic vascular circuits and may be helpful in monitoring the hemodynamics of these infants.

Analysis of Variance↗
PubMedDate not supplied

Pulmonary hypertension in children following extracorporeal membrane oxygenation therapy and repair of congenital diaphragmatic hernia.

OBJECTIVE: Pulmonary hypertension (PHT) is present in all children at birth, but its degree and rate of resolution in infants diagnosed with congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) need to be established. STUDY DESIGN: Twenty-one ECMO/CDH survivors (aged 3.2 +/- 1.4 years) were prospectively evaluated by Doppler echocardiography (ECHO) to determine the presence of PHT. Twenty children without structural heart disease were used as controls. Study patients received a physical examination and an electrocardiograph examination, and their charts were reviewed for neonatal course data. Patients found to have PHT by ECHO received a complete history and exercise treadmill/oxygen desaturation study. RESULTS: Eight of the 21 patients (38%) met echocardiographic criteria for PHT. No neonatal course data were found to be predictive of eventual PHT status. There was no correlation between physical examination or electrocardiographic findings and PHT. Complete histories showed five of the eight patients with PHT had some degree of exercise intolerance and seven had wheezing. Two of the seven patients studied on the treadmill desaturated 5% or greater from baseline. CONCLUSION: There is evidence that PHT either persists or recurs in a significant portion of the ECMO/CDH population and may remain symptomatic well beyond the neonatal period.

Child, Preschool↗