Right-sided Obstruction Research Articles

Painful ANA-Positive Scleroderma-Like Disease With Acral Ulcerations: A Case of Chronic Gangrenous Ergotism

Chronic ergotism is a rare cause of limb ischemia. In this case report, the authors present a 62-year-old woman with history of long-term use of ergotamine alkaloids for the treatment of menstrual pain, who developed a severe painful disease initially misdiagnosed as systemic sclerosis (scleroderma) for 3 decades. She presented with a combination of acral gangrene, foot ulcer, renal obstruction, mild pulmonary fibrosis, and reduced esophageal motility. Right-sided renal obstruction was evident. The condition was extremely painful and had led to muscular contractions and immobility, drug abuse, and anemia. After establishing the diagnosis of chronic gangrenous ergotism, changing drug therapy, mobilization, and treatment of chronic wounds, she showed a remarkable recovery. Eventually the foot ulcer was closed successfully using a mesh graft transplantation, and the patient was able to walk alone. Chronic ergotism is rare but has to be taken into account when presented with painful chronic digital and foot ulcers.

Right ventricular outflow tract obstruction after arterial switch operation for the Taussig–Bing heart☆

Incidence of right ventricular outflow tract obstruction (RVOTO) may be suspected to be higher after arterial switch operation (ASO) for Taussig-Bing heart than after ASO for transposition of the great arteries (TGA), as Taussig-Bing anomaly is frequently associated with aortic arch obstruction and subvalvular aortic stenosis. We evaluated the risk to develop RVOTO after ASO for Taussig-Bing heart. The 34 Taussig-Bing cases who underwent ASO from 1984 to 2005 were reviewed. RVOTO was defined as peak echo-gradient >or=30 mmHg across right ventricular outflow tract. Kaplan-Meier method was used to estimate time-related events. Subaortic stenosis was resected in 25 patients, 20 of whom (80%: 20/25) were discharged from hospital free from RVOTO. There was one early death: 2.9% mortality. Three patients died late. Actuarial survival was 85.1%+/-7.0% from 54 month onwards. Eleven survivors (36.7%: 11/30) experienced postoperative RVOTO. Obstruction was seen in 82% (9/11) of cases at subvalvular and/or valvular level. Surgery (n=4) or percutaneous intervention (n=2) was required in six patients. Patients discharged from hospital with RVOTO (n=8) were more likely to undergo reintervention for RVOTO (p=0.026). Freedom from reintervention for RVOTO decreased rapidly in the first two years to 86.5+/-6.3%, slowly thereafter (80.4+/-8.4% at year 7) and stabilized at 70.3+/-11.9% from year 11 on. Risk for RVOTO occurrence was 23.5+/-7.3% early after repair and progressively increased to level out at 53.6+/-11% at year 11. Patients who underwent subaortic resection were more likely (p=0.023) to be free from RVOTO occurrence or development. In the period under review, for patients who underwent ASO for simple (n=355) and complex (n=92) TGA, reoperation rate for neopulmonary stenosis was 0.3% (1/355) and 5.4% (5/92), respectively, to be compared to 11.8% (4/34) RVOTO rate of reoperation for Taussig-Bing heart in this study. Postoperative right-sided obstruction occurs more frequently after ASO repair of Taussig-Bing heart than after TGA arterial switching, leading to higher reintervention rate. Resection of the commonly associated subaortic stenosis often prevents RVOTO development.

Fetal Cardiac Tumors: Prenatal Diagnosis and Outcome

We present our experience in the management of fetuses diagnosed with huge cardiac tumors. These cases illustrate that the size of the tumor likely does not impact on survival as much as the location of the tumor and how it compromises blood flow into and out of the ventricles. We speculate that obstruction of right-sided inflow and/or simultaneous obstruction to outflow from both ventricles may lead to diminished cardiac output, atrial and caval hypertension, and hydrops fetalis. Obstruction can occur at any point in gestation and depends on both the size and the location of the tumor in relation to all cardiac structures. We therefore suggest serial assessment of these fetuses throughout gestation, particularly after the point of postnatal viability, to assess the hemodynamic effects that the tumor has on the heart. If obstruction to blood flow and/or early fetal compromise is noted, then the decision of whether to deliver early can be made. At the time of birth, if obstruction to blood flow persists, surgery can be considered, keeping in mind that the natural history of these tumors is to shrink and become clinically less important over time.

Gene Transfer of Prostaglandin Synthase Maintains Patency of the Newborn Lamb Arterial Duct

In congenital heart disease with left- or right-sided obstruction, prostaglandin E (PGE)1 or PGE2 is infused to maintain ductus arteriosus (DA) patency. We hypothesized that transfection of the DA with PGE synthase would lead to a greater production of PGE2 in situ and, hence, patency of the DA. The cDNA for human prostaglandin synthase was sequenced and ligated into a eukaryotic expression vector. The negative control was created by ligating the cDNA encoding the bacterial protein chloramphenicol acetyltransferase into the same plasmid. Transfection (600 microg DNA) was achieved in lambs within the first 24 h of life using the hemagglutinating virus of Japan (HVJ)-liposome transfection method with a custom-made, basket-weave-perforated catheter. Echocardiography was performed to assess DA patency until the time of sacrifice. To confirm expression of the transgene, PGE2 concentration was measured in organ culture of the DA by immunoassay and by Western immunoblotting of homogenized DA tissue. Patency of the DA was demonstrated by color Doppler in all the lambs (7/7) in which the PGE synthase was delivered, whereas functional closure was seen in the control group (6/6). The PGE2 concentration in the culture medium of the explanted DA in the treatment group was 3-fold higher than that of the control groups. Western immunoblotting confirmed the presence of PGE synthase in the treatment group. Gene transfer of PGE synthase to the DA is feasible and will maintain patency for at least 1 wk.

The effect of repair technique on postoperative right-sided obstruction in patients with truncus arteriosus

We reviewed our experience with repair of truncus arteriosus to assess the effect of type of right ventricular outflow tract reconstruction on perioperative morbidity, survival, and freedom from catheter-based interventions and reoperation. Patients undergoing repair of truncus arteriosus from June 1990 through February 2004 were evaluated on the basis of operative procedure regarding preoperative and postoperative variables, the need for postoperative catheter-based intervention or reoperation, and survival on the basis of univariate, multivariable, and actuarial analyses. Of 54 study patients, 15 (28%) received a valved homograft, and 39 (72%) received a direct connection with a variety of hood materials. Five (9.1%) patients died. Valved homograft recipients were more likely to require reoperation than patients receiving direct connections (40% vs 15%, P = .046); however, valved homograft and direct connection recipients had a similar incidence of the combined end point of reoperation or catheter-based intervention (40.0% vs 37.5%, P = .865). Univariate and multivariable modeling demonstrated use of valved homografts or direct connections with an autologous pericardial hood to be predictive of the need for later catheter-based intervention or reoperation. Actuarial analysis demonstrated a trend toward improved outcomes in the direct connection group and within the direct connection cohort, a statistically significant difference on the basis of hood type. Although the direct connection technique might not prevent later catheter-based intervention, it does reduce the need for reoperation. Outcomes among direct connection recipients were associated with hood type: polytetrafluoroethylene hoods (W. L. Gore & Associates, Inc, Tempe, Ariz) had the lowest rate of reintervention, and untreated autologous pericardial hoods had the highest rate of reintervention. We report excellent outcomes with primary repair of truncus arteriosus. Where anatomically appropriate, we advocate the direct connection technique.

Dermal choristoma of nasopharynx

The dermal choristoma of the nasopharynx is a developmental tumor that has been reported under a variety of different terms, including accessory auricle, hairy polyp, and dermoid. Although the majority of these lesions arise from the vault of the nasopharynx near the region of the eustachian tube orifice, they have also been reported to arise within the eustachian tube itself, from the nasopharyngeal surface of the soft palate, the oropharynx in the tonsillar region, and, rarely, in the middle ear and mastoid area. We report a case of an otherwise healthy 15-month-old female who presented with a 1-year history of right-sided clear rhinorrhea. Nasopharyngoscopy revealed a 1-cm firm, pinkish-white, smooth-surfaced, spherical, pedunculated mass arising from the left nasopharyngeal wall. The left-sided lesion acted as a ball valve to produce intermittent right-sided obstruction of the nasal passage. Imaging studies demonstrated a homogenous solid mass of the density of adipose tissue. It was excised without complication using cautery. Histopathologic examination revealed a solid mass of fibroadipose tissue that was surfaced by essentially normal skin. The keratinized stratified squamous epithelial surface exhibited a granular layer and numerous dermal appendage structures, including hair follicles, sebaceous glands, and sweat glands. The lesion did not exhibit elastic cartilage. These clinical and histologic findings support the diagnosis of dermal choristoma, a lesion that should be included in the differential diagnosis of nasopharyngeal masses in young individuals.

Emergency surgery for obstructing colorectal cancers: a comparison between right-sided and left-sided lesions

BACKGROUND: Fifteen to twenty percent of patients with primary colorectal cancers present with intestinal obstruction. Traditionally, different approaches have been used in the management of right-sided and left-sided colonic obstruction. Recently, single-stage resection with primary anastomosis in left colonic obstruction has been shown to have good results. The objective of this study was to compare the operative results of patients who had emergency operations for right-sided and left-sided obstructions from primary colorectal cancers. STUDY DESIGN: This is a retrospective study including 243 patients who underwent emergency operations for obstructing colorectal cancers from 1989 to 1997. Primary resection of the tumor-bearing segment followed by primary anastomosis was attempted when the conditions were feasible. The operative results of patients with right-sided tumors were compared with those of patients with left-sided tumors. RESULTS: One hundred seven patients had obstruction at or proximal to the splenic flexure (right-sided lesions), and 136 had lesions distal to the splenic flexure (left-sided lesions). The primary resection rate was 91.8%. Of the 223 patients with primary resection, primary anastomosis was possible in 197 patients. Among the 101 primary anastomoses in patients with left-sided obstruction, segmental resection with on-table lavage was performed in 75 patients and subtotal colectomy was performed in 26. The overall operative mortality rate was 9.4%, although that of the patients with primary resection and anastomosis was 8.1%. The anastomotic leakage rate for those with primary resection and anastomosis was 6.1%. There were no differences in the mortality or leakage rates between patients with right-sided and left-sided lesions (mortality: 7.3% versus 8.9%, p = 0.79; leakage: 5.2% versus 6.9%, p = 0.77). Colocolonic anastomosis did not show a significant difference in leakage rate when compared with ileocolonic anastomosis (6.1% versus 6.0%, p = 1.0). CONCLUSIONS: This study showed that primary resection and anastomosis for left-sided malignant obstruction, either by segmental resection with on-table lavage or subtotal colectomy, was not more hazardous than primary anastomosis for right-sided obstruction. The single-stage procedure should be the objective for the treatment of patients with obstructing colorectal cancers, except when patients are hemodynamically unstable during surgery or when the condition of the bowel is not optimal for primary anastomosis.

Surgery for bilateral outflow tract obstruction in elastin arteriopathy

Objective: A number of patients with Williams syndrome or other forms of elastin arteriopathy have stenoses of pulmonary arteries in addition to supravalvular aortic stenosis. We sought to investigate the effect of the degree of pulmonary arterial stenosis on the prognosis after an operation for supravalvular aortic stenosis to help define the optimal treatment strategy for patients with severe forms of elastin arteriopathy. Methods: Between 1960 and 1999, 33 patients underwent operations for supravalvular aortic stenosis while having significant stenoses of the pulmonary arteries. We retrospectively reviewed patient charts, obtained current follow-up information, and determined risk factors for survival and reoperation. Results: Fifteen patients with moderate right-sided obstructions (confirmed by pulmonary artery Z-scores and right ventricular/descending aortic pressure ratio) underwent operations for supravalvular aortic stenosis only. Eighteen patients had more severe right-sided obstructions and underwent surgical relief of pulmonary arterial stenoses or right ventricular outflow tract obstruction in addition to operations for supravalvular aortic stenosis. Eight patients had undergone preoperative balloon dilations of stenotic pulmonary arteries. There were 6 early deaths and 1 late death in our series. Survival at 10 and 20 years was 76% (70% confidence interval, 68%-84%) and freedom from reintervention was 59% (70% confidence interval, 46%-71%) at 10 years and 49% (70% confidence interval, 35%-62%) at 20 years. Multivariate analysis revealed that patients with a right ventricular/descending aortic pressure ratio of 1.0 or more were at higher risk for reintervention but not for death. Conclusions: Surgical treatment of pulmonary artery obstructions in elastin arteriopathy is palliative but, in conjunction with balloon dilation of peripheral pulmonary arteries, offers good long-term survival to patients with the severest form of elastin arteriopathy. (J Thorac Cardiovasc Surg 2000;120:755-63)

Outflow obstruction after the arterial switch operation: A multiinstitutional study

Objective: Our objectives were to discover whether outflow obstruction immutably accompanies the arterial switch operation and to identify factors that may decrease its prevalence. Methods: Percutaneous or surgical reintervention for obstruction after an arterial switch was selected as an end point for obstruction. Its risk factors were identified by time-related multivariable analyses of yearly follow-up data from 514 neonates with simple transposition or transposition with ventricular septal defect entering 23 institutions before 15 days of age between January 1, 1985, and March 1, 1989. Results: Sixty-two patients underwent 86 reinterventions for right-sided obstruction (83% free at 10 years) and six for left-sided obstruction (98% free at 10 years). After 2 years, right-sided obstruction occurred at a rate of about 1% per year and left-sided at a rate of about 0.1% per year. Right ventricular infundibular or valvular obstruction was associated with the aorta and pulmonary trunk positioned side-by-side, coexisting coarctation, use of prosthetic material in sinus reconstruction, one institution, and earlier institutional experience. Pulmonary trunk or pulmonary artery obstruction was associated with lower birth weight, left coronary artery arising from sinus 2, coronary explantation away from the transection site, three institutions, and earlier institutional experience. Conclusions: A risk-adjusted base incidence (0.5% per year) of reintervention for right-sided obstruction continues late after operation. It is due in part to congenital variability or abnormality of right ventricular outflow structures and to experience and surgeon variability resulting in suboptimal pulmonary trunk reconstruction. The same sources of variability probably affect the aortic root, but its native characteristics plus higher distending pressure make the base incidence considerably less (0.1% per year). (J Thorac Cardiovasc Surg 1997;114:975-90)

Pregnancy in patients with pulmonary autograft valve replacement.

The purpose of this study is to determine the outcome and complications of pregnancy in women with pulmonary autograft valve replacement for aortic valve disease. The records of all women who had undergone pulmonary autograft valve replacement at the National Heart Hospital (now Royal Brompton Hospital) since 1968 were reviewed. From 1968 to 1993, 27 hospital survivors were female and among eight of them there were 14 pregnancies. All women were in Ability Index 1 at time of pregnancy with normal ventricular function, mild aortic regurgitation (six), mild pulmonary regurgitation (three) and mild pulmonary stenosis (two). None took anticoagulants. There was no maternal death, thromboembolic or haemorrhagic event or evidence of deterioration in valve function during pregnancy. Except for one woman (Ability Index 3) who developed dilated cardiomyopathy without aortic or pulmonary valve disease 6 months after delivery, the women remained in Ability Index 1 after pregnancy. There was no significant progression of aortic regurgitation (mild after seven pregnancies), pulmonary regurgitation (mild after six) or right-sided obstruction (mild after four). Reoperation for right-sided obstruction was carried out in two patients 4 and 7 years after a second pregnancy (9 and 15 years after the pulmonary autograft). No valve-related complications occurred during pregnancy and pregnancy appeared to have no effect on the function of the pulmonary valve autograft or the right-sided homograft. The pulmonary autograft is thus an ideal procedure for a young female needing aortic valve replacement.

Spectrum of cardiovascular anomalies in Williams-Beuren syndrome

This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2:1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticists. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10-40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse.(ABSTRACT TRUNCATED AT 250 WORDS)

Noninvasive diagnosis of right-sided extracardiac conduit obstruction by combined magnetic resonance imaging and continuous-wave Doppler echocardiography

Right-sided extracardiac conduits are frequently complicated by obstruction over time. We compared the utility of two-dimensional and Doppler echocardiography and magnetic resonance imaging in the diagnosis of postoperative right-sided obstruction with cardiac catheterization and angiography in 10 patients with xenograft or homograft conduits. Correlation (r = 0.95) between continuous-wave Doppler estimates and catheter pullback pressure gradients across the conduits was excellent. Echocardiography could only visualize five of 10 conduits in their entirety. Magnetic resonance imaging visualized all conduits and showed statistically significant (kappa = 0.58) agreement with angiography in the localization and estimation of severity of a variety of right-sided obstructions in these patients. However, flow voids created by the metallic ring around xenograft valves led to a false negative diagnosis of valvular stenosis in four patients when magnetic resonance imaging was used alone. Doppler studies correctly indicated obstruction in these patients. The combination of magnetic resonance imaging studies and continuous-wave Doppler echocardiography can be useful to noninvasively evaluate right-sided obstruction in postoperative patients with right-sided extracardiac conduits.

Complete Atrioventricular Canal and Tetralogy of Fallot: Surgical Considerations

A review of 13 autopsy specimens and of 13 patients who were operated on for complete atrioventricular (AV) canal and tetralogy of Fallot between 1975 and 1985 revealed a number of anatomical details that are important in the successful repair of this combined lesion. A bridging anterior leaflet was present in 25 hearts. A septum primum was present and attached to leaflet tissue in 13. In all 26, a ventricular septal communication was present beneath the bridging anterior leaflet and extended anteriorly, but in 14 there was no ventricular septal defect underneath the posterior leaflet. Additional pathological features included the following: leaflet tissue deficiency (4 hearts), single left papillary muscle (3), accessory valve orifice (4), and left ventricular (4) or right ventricular (RV) (1) dominance. All 26 had infundibular stenosis, and 10 had hypoplastic pulmonary annuli. One had pulmonary atresia, and 6 had branch pulmonary artery stenosis. Surgical technique was modified to include incision of the septum primum in 7. Because of rightward displacement of the anterior ventricular septum and also to minimize the risk of causing subaortic stenosis, the bridging anterior leaflet was divided more toward the tricuspid orifice so as to parallel the crest of the ventricular septum. Transannular RV outflow patches were used in 10 patients, and a right ventricle–pulmonary artery conduit was placed in 1 patient. Three required repair of branch pulmonary artery stenosis. There were no hospital deaths. Three patients died late of residual AV valve regurgitation and branch pulmonary artery stenosis (2) and sepsis (1). One patient with RV hypoplasia and postoperative tricuspid regurgitation had a Fontan operation. Four patients required left AV valve replacement (3 early and 1 late). The AV canal component in complete AV canal with tetralogy of Fallot is more complex than in isolated complete AV canal. Also, in addition to infundibular stenosis, other right-sided obstructions are common. A better understanding of the many anatomical variables has led to improvement in surgical results.

Complete transposition of the great arteries: Visualization of left and right outflow tract obstruction by oblique subcostal two-dimensional echocardiography

Subcostal oblique 2-dimensional echocardiography was performed in 64 infants younger than 2 years with complete transposition of the great arteries (TGA) (situs solitus, concordant atrioventricular and discordant ventriculoarterial connections). All patients examined before cardiac catheterization had a correct diagnosis by 2-dimensional echocardiography using the subcostal oblique views. Twelve patients had associated left ventricular (LV) outflow tract obstruction and 7 had right ventricular (RV) outflow obstruction. The standard parasternal views failed to diagnose obstruction in 1 patient with LV outflow obstruction and 5 with RV outflow obstruction; the subcostal left oblique cut and long axis of the left ventricle visualized all left-sided obstructions, and right-sided obstructions were correctly displayed in 5 of 7 cases using a combination of left oblique and right oblique cuts. Two-dimensional echocardiographic subcostal oblique views allow an excellent definition of the morphologic characteristics of RV and LV outflow tracts in patients with TGA and improve the diagnosis of the outflow obstruction in these malformations.

Ventricular septal defect and subaortic stenosis: An analysis of 41 patients

Forty-one patients with subaortic stenosis (SAS) and ventricular septal defect (VSD) were identified from the cardiac records of the Hospital for Sick Children, Toronto, Ontario. The diagnosis of an associated SAS was made clinically in only 1 patient, who had findings of left ventricular (LV) hypertrophy with strain on the electrocardiogram. There was a delay of 3.1 years between initial presentation and detection of SAS. The SAS was not diagnosed at initial catheterization in 17 patients and was confirmed at subsequent catheter studies in 8 patients, surgery in 5 and autopsy in 4. Associated defects included coarctation of the aorta in 12 patients, mitral valve abnormalities in 4, and right-sided obstructions, including anomalous right ventricular muscle bundles in 6 patients, tetralogy in 4 and pulmonic stenosis in 1 patient. The mean gradient across the LV outflow tract was 25 mm Hg. Nineteen patients had serial catheters without intervening surgery, and the outflow gradient increased from a mean of 9 to 36 mm Hg. The mechanism of SAS consisted of fibrous diaphragm and fibromuscular obstruction in 31 cases, muscular narrowing in 4, protruding tricuspid valve leaflet in 2, hypertrophic cardiomyopathy in 2, anterolateral twist in 1 patient and redundant tissue tag in 1. Thirty-eight patients had a perimembranous VSD, 19 of whom had an associated so-called aneurysm of the membranous septum; 2 had an infundibular VSD and 1 patient had a central muscular defect. Although the SAS was located below the VSD in 30 cases, the associated heart failure and reduced cardiac output can mask the presence or severity of associated SAS. Cross-sectional echocardiography and axial angiography have greatly improved recognition of associated SAS.