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Biomedical subjects

V M Reddy

Publications and source records attributed to V M Reddy.

At least 19 recordsLinked to original sources
PubMed2006

Neurologic events in neonates treated surgically for congenital heart disease.

OBJECTIVE: The incidence of acute neurologic events prior to discharge in neonates with congenital heart disease (CHD) was determined and peri-operative characteristics predictive of a neurologic event were identified. STUDY DESIGN: A retrospective chart review over 1 year was conducted of infants <1 month of age with a diagnosis of CHD. Outcomes were measured by the occurrence of an acute neurologic event defined as electroencephalogram (EEG)-proven seizure activity, significant hypertonia or hypotonia, or choreoathetosis prior to hospital discharge. Stepwise logistic regression identified variables most likely to be associated with an acute neurologic event. RESULTS: Surgical intervention occurred in 95 infants who were admitted with a diagnosis of CHD. The survival rate was 92%. Of the survivors, 16 (17%) had an acute neurologic event, with 19% of events occurring preoperatively. Factors associated with neurologic events included an elevated nucleated red blood cell (NRBC) count, an abnormal preoperative brain imaging study, and a 5-min Apgar score <7 (P<0.05). CONCLUSIONS: Neonates with CHD have a significant risk of neurologic events. Preoperative brain imaging, the 5-min Apgar score, and initial serum NRBC counts may identify infants at highest risk for central nervous system injury.

Acute Disease↗
PubMed2006

Is admission for epistaxis more common in Caucasian than in Asian people? A preliminary study.

OBJECTIVES: Epistaxis is a common ENT complaint. Although casual observation suggested that it is more common in Caucasian, compared with Asian people, a literature search failed to find any studies investigating ethnicity and epistaxis. The aim of this study was to identify any differences in emergency admission rates for epistaxis between Asian and Caucasian people. DESIGN: Retrospective observational study using hospital computerised data (HISS). SETTING: Large University Hospital accepting ENT emergencies. PARTICIPANTS: All Asian and Caucasian patients admitted under ENT care as an emergency (1 January 2000 to 30 November 2005), split into two groups: one composed of epistaxis patients, the other of all other ENT emergency admissions. MAIN OUTCOMES MEASURES: The proportions of Asian and Caucasian patients among the two patient groups, either epistaxis admissions or other ENT emergency admissions. RESULTS: The proportions of Asian and Caucasian patients in the group admitted with emergency epistaxis were 7.1% (100/1410) and 92.9% (1310/1410) respectively. However, the proportions of Asian and Caucasian patients in the group composed of any other ENT emergency were 13.2% (729/5515) and 86.8% (4786/5515), respectively (chi-squared P < 0.01). CONCLUSIONS: Caucasian people form an unexpectedly large, and Asians a smaller proportion of emergency epistaxis admissions. The possibility of an ethnic risk factor for epistaxis warrants further investigation.

Asian People↗
PubMed2004

Mycobacterium avium binds to mouse intestinal mucus aldolase.

SETTING: Mycobacterium avium complex (MAC) is known to colonize the gastrointestinal tract of human immunodeficiency virus (HIV) infected patients before causing bacteremia and disseminated disease. However, the mechanism involved in the gastrointestinal colonization is not known. OBJECTIVE: To identify putative intestinal mucus receptors which serve as anchor for MAC colonization. DESIGN: C57BL/6 mouse intestinal mucus was subjected to single and two-dimensional electrophoresis and blotted on nitrocellulose membranes. MAC specific mucus proteins were identified by probing the mucus western blots with biotinylated proteins derived from M.avium strain 101 (MAC101). RESULTS: Biotinylated MAC 101 proteins recognized a 39 kDa intestinal mucus glycoprotein. The protein displaying an isoelectric point (pI) of 9.0, was found to be periodate sensitive but resistant to sialidase, heparinase I and chondroitinase ABC. The internal amino acid sequence of the 39 kDa protein displayed homology with fructose-1-6-bisphosphate aldolase B (aldolase). The proclivity between MAC adhesins and aldolase was confirmed by probing rabbit muscle aldolase with MAC proteins. Furthermore, both 25 and 31 kDa MAC adhesins, superoxide dismutase and heparin binding protein, respectively, were found to bind to aldolase. CONCLUSIONS: MAC binds to intestinal mucus aldolase, conceivably facilitating intestinal colonization of the organism.

Amino Acid Sequence↗
PubMed2002

Interaction of Mycobacterium tuberculosis with human respiratory epithelial cells (HEp-2).

SETTING: Mycobacterium tuberculosis (MTB) is known to adhere to, invade, multiply inside, and modulate respiratory epithelial cell functions. However, the mode and the molecules involved in the interaction of MTB with respiratory cells are not known. OBJECTIVE: To study post-adherence morphological changes in human respiratory epithelial cells and identify MTB components involved in the binding to these cells. DESIGN: The mechanism of interaction of MTB human respiratory epithelial cells (HEp-2) was investigated by incubating MTB with HEp-2 cells, whereupon the morphological changes were examined under scanning electron microscopy. MTB components involved in the interaction with HEp-2 cells were identified by probing Western blots of mycobacterial sonicates with biotinylated HEp-2 extracts. RESULTS: MTB readily bound to the surface of HEp-2 cells. Adherence of tubercle bacilli on the surface caused membrane perturbation resulting in increased surface membrane projections, particularly towards the margins of the cells. Long thin epithelial membrane projections formed loops around the bacilli holding them on the surface. Membrane projections fused to form a web in which the bacilli were entrapped. Concomitant membrane cavitations seen beneath the site of attachment of the bacilli conceivably caused internalization of the organisms. At least five MTB proteins ( approximately 112, 35, 28, 21 and 15 kDa) specific for HEp-2 cells were identified. Of these, the 28 kDa protein was the predominant HEp-2-binding protein and was found to be heparin-binding hemagglutinin (HBHA). While M. bovis BCG strain showed the same five HEp-2-binding proteins, M. smegmatis, displayed only one (31 kDa) HEp-2-specific protein. CONCLUSION: MTB binds to HEp-2 cells through multiple proteins, and the binding causes membrane perturbations that result in internalization of the organisms.

Bacterial Adhesion↗
PubMed2001

Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome.

BACKGROUND: Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome. METHODS AND RESULTS: We reviewed patients with HLHS between July 1992 and March 1999 to determine the influence of prenatal diagnosis on preoperative clinical status, outcomes of stage 1 surgery, and parental decisions regarding care. Of 88 patients, 33 were diagnosed prenatally and 55 after birth. Of 33 prenatally diagnosed patients, 22 were live-born, and pregnancy was terminated in 11. Of 22 prenatally diagnosed patients who were live-born, 14 underwent surgery, and parents elected to forego treatment in 8. Of 55 patients diagnosed postnatally, 38 underwent surgery, and 17 did not because of parental decisions or clinical considerations. Prenatally diagnosed patients were less likely to undergo surgery than patients diagnosed after birth (P:=0.008). Among live-born infants, there was a similar rate of nonintervention. Among patients who underwent surgery, survival was 75% (39/52). All patients who had a prenatal diagnosis and underwent surgery survived, whereas only 25 of 38 postnatally diagnosed patients survived (P:=0.009). Patients diagnosed prenatally had a lower incidence of preoperative acidosis (P:=0.02), tricuspid regurgitation (P:=0.001), and ventricular dysfunction (P:=0.004). They were also less likely to need preoperative inotropic medications or bicarbonate (P:=0.005). Preoperative factors correlating with early mortality included postnatal diagnosis (P:=0.009), more severe acidosis (P:=0.03), need for bicarbonate or inotropes (P:=0.008 and 0.04), and ventricular dysfunction (P:=0.05). CONCLUSIONS: Prenatal diagnosis of HLHS was associated with improved preoperative clinical status and with improved survival after first-stage palliation in comparison with patients diagnosed after birth.

Age Factors↗
PubMed2001

Perioperative administration of angiotensin converting enzyme inhibitors decreases the severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis.

BACKGROUND: Pleural effusions after bidirectional cavopulmonary anastomosis remain a significant cause of morbidity. Prolonged effusions in such patients have been associated with persistent elevations in plasma renin and angiotensin II. METHODS: We conducted a controlled study in 36 patients (median age 8 months) undergoing bidirectional cavopulmonary anastomosis. Enalapril (5 mcg/kg) was administered intravenously within 1 hour of surgery and every 12 hours thereafter in 18 patients; when these patients were tolerating feeds, enalapril was switched to enteral captopril (3 mg/kg/day) every 8 hours. The other 18 patients did not receive perioperative angiotensin converting enzyme inhibitors. Using standardized criteria for discontinuation of chest tubes (< 2 mL/kg/day), volume and duration of pleural drainage were compared between groups. RESULTS. There were no differences between groups in demographic, diagnostic, or hemodynamic factors. There was no difference in cardiopulmonary bypass time between groups and no difference in postoperative pulmonary arterial pressures. The duration of pleural drainage was shorter (2.2+/-1.4 vs 5.9+/-1.4 days, p<0.001) and the volume less during the first 24 hours (4.7+/-1.2 vs 7.7+/-2.1 mL/kg, p<0.001) and overall (10.6+/-2.4 vs 19.6+/-4.5 mL/kg, p<0.001) in patients who received angiotensin converting enzyme inhibitors than those who did not. Readmission for persistent effusions was required in 3 patients who did not receive angiotensin converting enzyme inhibitors and none who did (p=0.11). CONCLUSIONS: Perioperative administration of angiotensin converting enzyme inhibitors is associated with decreased severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis.

Angiotensin-Converting Enzyme Inhibitors↗
PubMed2001

The Ross procedure in children and young adults: a word of caution.

BACKGROUND: Disease of the aortic valve in children and young adults is a complex entity whose management is the subject of controversy. The Ross and the Ross-Konno procedures have become the primary choices for aortic valve replacement in children because of growth potential, optimal hemodynamic performance, and lack of the need for anticoagulation. However, concern persists regarding the longevity of the pulmonary autograft, especially in patients with aortic insufficiency. METHODS: Between June 1993 and February 2000, 72 Ross and Ross-Konno procedures were performed at our institution: 81% of the patients were less than 15 years old. Preoperative, postoperative, and follow-up clinical, echocardiographic, and hemodynamic data were reviewed. Statistical analysis was performed to identify the risk factors for deteriorating autograft function. RESULTS: Aortic insufficiency was an indication for the Ross procedure in 17 patients and mixed lesions with predominant aortic insufficiency in 10. Of the 45 other patients, 32 had aortic stenosis and 13 had mixed lesions with predominant aortic stenosis. There were no deaths during a follow-up of 5 to 80 months. Autograft reoperation was necessary in the follow-up period in 7 patients for severe aortic insufficiency. Moderate insufficiency was identified in 5 additional patients. Aortic insufficiency or predominant aortic insufficiency, as a preoperative hemodynamic indication for the Ross procedure, reached statistical significance (P =.031) as a risk factor for autograft failure. CONCLUSION: The Ross and the Ross-Konno procedures have changed the prognosis of children and young adults with complex aortic valve disease. However, the Ross procedure should be performed with caution in older children in whom aortic insufficiency is a preoperative hemodynamic indication. Further follow-up to delineate the risk factors for autograft dysfunction in children and young adults is necessary to better define the indications for the Ross procedure.

Adolescent↗
PubMed2001

A prospective randomized study comparing volume-standardized modified and conventional ultrafiltration in pediatric cardiac surgery.

BACKGROUND: Modified ultrafiltration has been touted as superior to conventional ultrafiltration for attenuating the consequences of hemodilution after cardiac surgery with cardiopulmonary bypass in children. We conducted a prospective randomized study to test the hypothesis that modified and conventional ultrafiltration have similar clinical effects when a standardized volume of fluid is removed. METHODS: From October 1998 to September 1999, 110 children weighing 15 kg or less (median weight 6.1 kg, median age 6.3 months) undergoing surgery with cardiopulmonary bypass for functionally biventricular congenital heart disease were randomized to conventional (n = 67) or arteriovenous modified ultrafiltration (n = 43) for hemoconcentration. The volume of fluid removed with both methods was standardized as a percentage of effective fluid balance (the sum of prime volume and volume added during cardiopulmonary bypass minus urine output): in patients weighing less than 10 kg, 50% of effective fluid balance was removed, whereas 60% was removed in patients weighing 10 to 15 kg. Hematocrit, hemodynamics, ventricular function, transfusion of blood products, and postoperative resource use were compared between groups. RESULTS: There were no significant differences between groups in age, weight, or duration of cardiopulmonary bypass. The total volume of fluid added in the prime and during bypass was greater in patients undergoing conventional ultrafiltration than in those receiving modified ultrafiltration (205 +/- 123 vs 162 +/- 74 mL/kg; P =.05), although the difference was due primarily to a greater indexed priming volume in patients having conventional ultrafiltration. There was no difference in the percentage of effective fluid balance that was removed in the 2 groups. Accordingly, the volume of ultrafiltrate was greater in patients receiving conventional than modified ultrafiltration (95 +/- 63 vs 68 +/- 28 mL/kg; P =.01). Preoperative and postoperative hematocrit levels were 35.6% +/- 6.6% and 36.3% +/- 5.6% in patients having conventional ultrafiltration and 34.4% +/- 6.7% and 38.7% +/- 7.5% in those having modified ultrafiltration. By repeated-measures analysis of variance, patients receiving modified and conventional ultrafiltration did not differ with respect to hematocrit value (P =.87), mean arterial pressure (P =.85), heart rate (P =.43), or left ventricular shortening fraction (P =.21) from baseline to the postbypass measurements. There were no differences between groups in duration of mechanical ventilation, stay in the intensive care unit, or hospitalization. CONCLUSIONS: When a standardized volume of fluid is removed, hematocrit, hemodynamics, ventricular function, requirement for blood products, and postoperative resource use do not differ between pediatric patients receiving conventional and modified ultrafiltration for hemoconcentration after cardiac surgery.

Blood Transfusion↗
PubMed2001

Superior cavopulmonary anastomosis suppresses the activity and expression of pulmonary angiotensin-converting enzyme.

BACKGROUND: Superior cavopulmonary anastomosis is widely used for palliation of various forms of univentricular heart defects. However, clinically significant pulmonary arteriovenous malformations develop in 15% to 25% of patients after surgery. OBJECTIVE: To assess altered regulation of pulmonary vascular tone caused by superior cavopulmonary anastomosis in an ovine model. METHODS: Lambs, aged 35 to 45 days, underwent an end-to-end anastomosis of the superior vena cava to the right pulmonary artery. In age-matched controls, a sham operation was performed. Arteriovenous malformations were detectable by contrast echocardiography by 8 weeks after surgery. Animals (n = 24) were studied at various time points after the operations. Expression of angiotensin-converting enzyme messenger RNA, protein levels, and enzyme activity were measured in lung homogenates. Levels of angiotensin II were measured by enzyme-linked immunosorbent assay. RESULTS: Expression of angiotensin-converting enzyme messenger RNA and protein was significantly reduced at 1 to 5 weeks after superior cavopulmonary anastomosis. Angiotensin-converting enzyme activity in the right lung of animals subjected to superior cavopulmonary anastomosis was reduced 86% +/- 1% (standard deviation) compared with control values at 1 week (P =.003) and 77% +/- 8.5% at 2 weeks (P <.001) after surgery. This correlated with a 59% +/- 3.5% (P =.007) reduction in angiotensin II levels up to 5 weeks after cavopulmonary anastomosis. By 15 weeks after the operations, angiotensin II levels were equivalent to control levels (P =.19). CONCLUSIONS: Superior cavopulmonary anastomosis causes an early reversible reduction in activity and expression of angiotensin-converting enzyme, resulting in decreased circulating levels of the vasoconstrictor angiotensin II. These results suggest that the ability of the pulmonary endothelium to regulate vascular tone is inhibited after superior cavopulmonary anastomosis. Dilation of the affected vasculature induced by cavopulmonary anastomosis may contribute to the disordered vascular remodeling observed in this setting.

Angiotensin II↗
PubMed2001

Hemidiaphragmatic paralysis increases postoperative morbidity after a modified Fontan operation.

OBJECTIVES: After a Fontan procedure, forward pulmonary blood flow is augmented during inspiration because of negative intrathoracic pressure. Total pulmonary blood flow is higher during inspiration. With hemidiaphragmatic paralysis, inspiratory augmentation of pulmonary flow is lost or diminished. The objective of this study was to compare early postoperative morbidity after the modified Fontan operation in patients with and without hemidiaphragmatic paralysis. METHODS: A case-control analysis was performed comparing 10 patients with documented hemidiaphragmatic paralysis against 30 patients without paralysis who were matched for diagnosis, fenestration, and age. The following early postoperative outcomes were assessed: duration of ventilator support, duration of hospital stay, incidence of ascites, prolonged effusions, and readmission. RESULTS: Preoperatively, there were no significant differences between the 2 groups. However, among the postoperative outcomes, the duration of hospital stay (25.4 +/- 16.6 days vs 10.8 +/- 6.3 days; P =.03), incidence of ascites (70% vs 3%; P <.001), prolonged pleural effusions (60% vs 13%; P =.007), and readmission (50% vs 7%; P =.007) were significantly greater in patients with hemidiaphragmatic paralysis than in those without hemidiaphragmatic paralysis. CONCLUSIONS: Hemidiaphragmatic paralysis after the modified Fontan operation is associated with an increase in early morbidity. Care should be taken to avoid injury to the phrenic nerve. Patients with prolonged effusions should be evaluated for hemidiaphragmatic paralysis.

Case-Control Studies↗
PubMed2001

A new flavone glycoside, 5-hydroxy 7,3',4',5'-tetra-methoxyflavone 5-O-beta-D-xylopyranosyl-(1-->2)-alpha-L-rhamnopyranoside from Bauhinia variegata Linn.

A new flavone glycoside m.f. C(30)H(36)O(15) m.p. 252-253 degrees C, [M]+ 636 (EIMS) was isolated from the acetone soluble fraction of the concentrated 95% ethanolic extract of the seeds of Bauhinia variegata (Linn). It was identified as 5-hydroxy7,3',4',5'-tetra-methoxyflavone 5-O-beta-D-xylopyranosyl-(1-->2)-alpha-L-rhamnopyranoside (1) by various colour reactions, chemical degradations and spectral techniques.

Aspartic Acid Endopeptidases↗
PubMed2001

The safety and efficacy of sevoflurane anesthesia in infants and children with congenital heart disease.

UNLABELLED: We tested the hypothesis that sevoflurane is a safer and more effective anesthetic than halothane during the induction and maintenance of anesthesia for infants and children with congenital heart disease undergoing cardiac surgery. With a background of fentanyl (5 microg/kg bolus, then 5 microg. kg(-1). h(-1)), the two inhaled anesthetics were directly compared in a randomized, double-blinded, open-label study involving 180 infants and children. Primary outcome variables included severe hypotension, bradycardia, and oxygen desaturation, defined as a 30% decrease in the resting mean arterial blood pressure or heart rate, or a 20% decrease in the resting arterial oxygen saturation, for at least 30 s. There were no differences in the incidence of these variables; however, patients receiving halothane experienced twice as many episodes of severe hypotension as those who received sevoflurane (P = 0.03). These recurrences of hypotension occurred despite an increased incidence of vasopressor use in the halothane-treated patients than in the sevoflurane-treated patients. Multivariate stepwise logistic regression demonstrated that patients less than 1 yr old were at increased risk for hypotension compared with older children (P = 0.0004), and patients with preoperative cyanosis were at increased risk for developing severe desaturation (P = 0.049). Sevoflurane may have hemodynamic advantages over halothane in infants and children with congenital heart disease. IMPLICATIONS: In infants and children with congenital heart disease, anesthesia with sevoflurane may result in fewer episodes of severe hypotension and less emergent drug use than anesthesia with halothane.

Anesthetics, Inhalation↗
PubMed2001

Cardiac surgery for premature and low birth weight neonates.

Congenital heart defects in low birth weight infants are typically managed with supportive therapy or palliative surgery and definitive repair is delayed. However, the morbidity of this approach has been shown to be high. At the University of California San Francisco, our approach to these infants has been one of complete repair in the neonatal period or as early as possible. Since 1992, 116 neonates and infants under 2,500 g underwent complete repair of simple and complex cardiac defects using cardiopulmonary bypass and circulatory arrest was avoided in the majority. Actuarial survival was 80% at 1 year. Noncardiac abnormalities contribute significantly to perioperative and late morbidity and mortality. Reintervention rates are comparable to other neonates. Our data suggests that complete repair of simple and complex congenital heart defects can be performed safely and effectively in premature and low birth weight infants.

Cardiopulmonary Bypass↗
PubMed2001

Techniques to avoid circulatory arrest in neonates undergoing repair of complex heart defects.

Advances in cardiopulmonary bypass and cardiac surgical techniques have tremendously improved the outlook for children born with congenital heart defects. However, cardiopulmonary bypass, hypothermia, and circulatory arrest are all unphysiologic states and are associated with some morbidity, and the most significant are neurologic sequelae, which can be subtle or devastating. Circulatory arrest, often used as an adjunct to deep hypothermia, is a major contributing factor to the neurologic complications after cardiopulmonary bypass. In the last decade, techniques to avoid circulatory arrest have been introduced with a substantial decrease in neurologic sequelae.

Cardiopulmonary Bypass↗
PubMed2000

Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients.

BACKGROUND: Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. METHODS AND RESULTS: Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients. CONCLUSIONS: Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

Aorta↗
PubMed2000

Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect.

BACKGROUND: The frequency, distribution, and surgical importance of coronary artery to pulmonary artery (CAPA) collaterals have not been established. The aim of this study was to establish prevalence, anatomical pattern, and significance of CAPA in patients with pulmonary atresia and ventricular septal defect (PA/VSD). METHODS: We reviewed cardiac catheterization and operative data of 87 consecutive, unselected patients who underwent one-stage complete unifocalization for PA/VSD and assessed major systemic to pulmonary collaterals from July 1992 to June 1998. RESULTS: CAPA collaterals were diagnosed in 9 of 87 patients (10%). The collaterals originated from the left coronary artery system in 7 patients and the right in 2. Collaterals from the left coronary system arose from the left main coronary artery in 3 patients and the circumflex in 4. All collaterals joined the central pulmonary artery, which bifurcated and supplied both lungs. One collateral from the right coronary system joined the stump of the main pulmonary artery and the other gave origin to a true left pulmonary artery, which was the sole supply to 75% of the left lung. Coronary artery enlargement was seen in 2 patients only. No patient had evidence of myocardial ischemia. Coronary collaterals comprised a dual source of pulmonary blood flow in all but 1 patient. During unifocalization, the CAPA collaterals were ligated at its origin in all cases, and the collateral from the right coronary to the left pulmonary artery was unifocalized. CONCLUSIONS: The prevalence of CAPA collaterals in patients with PA/VSD is approximately 10%. The diagnosis may be missed without appropriate angiograms. We recommend selective ascending aortogram or selective coronary angiogram in all patients.

Adolescent↗
PubMed2000

Double-chambered right ventricle presenting in adulthood.

BACKGROUND: Double-chambered right ventricle is a form of right ventricular outflow tract obstruction that develops over time, often in patients with an abnormally short distance between the moderator band and pulmonary valve. This lesion typically presents in childhood or adolescence and is often accompanied by a ventricular septal defect. Only a handful of previous cases have been described in which double-chambered right ventricle occurred in adulthood. METHODS: Since 1992, three patients more than 30 years old (38, 43, and 66 years of age) have presented at our institution with unusual symptoms or a previous incorrect diagnosis. We reviewed the clinical data in these patients. RESULTS: Presenting symptoms included syncope, angina, and severe dyspnea resembling pulmonary hypertension. In 1 patient, disease was categorized as New York Heart Association class IV, and in the other 2 as class III. Coexisting anomalies included a patent foramen ovale or secundum atrial septal defect in 2 patients, a small ventricular septal defect in 1 (with a probable history of ventricular septal defect in another), and mild aortic regurgitation in 1. All patients required urgent or emergent operations, with peak pressures in the proximal right ventricular chamber of 135 to 180 mm Hg and severely depressed left ventricular function in 1 patient. Resection of the anomalous right ventricular muscle bundles was achieved through a right atrial approach in all patients. All patients were alive with improved functional status at follow-up, which was between 15 and 40 months. CONCLUSIONS: Right ventricular outflow tract obstruction resulting from a double-chambered right ventricle is rare in adults, but when it does occur it can present with unusual symptoms. When evaluating the patient with signs or symptoms of primary right heart failure, cardiologists should make an effort to image the entire right heart complex. Subcostal echocardiography can facilitate adequate visualization of the right ventricle when it is difficult to distinguish the subpulmonary outflow tract from the parasternal and apical windows.

Adult↗