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Primary cardiac tumours are rare and frequently only diagnosed post-mortem. Recent improvements in non-invasive imaging techniques have led to an increasing number being diagnosed in life, allowing surgical resection. We report a primary leiomyosarcoma presenting with obstruction of the left ventricular outflow tract.
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BACKGROUND: This study evaluates the results of the arterial switch operation for early total repair of double-outlet right ventricle with subpulmonary ventricular septal defect (the Taussig-Bing heart). METHODS: From 1986 through April 2003, 27 patients with Taussig-Bing anomaly underwent arterial switch operation. Twenty patients were neonates (n = 11) or infants younger than 3 months (n = 9). Obstruction of aortic arch (n = 19) or subaortic right ventricular outflow tract obstruction (n = 20) and unusual coronary artery patterns (n = 19) were common. Total correction as a single procedure was performed in 21 patients. Events are depicted by Kaplan-Meier curves. RESULTS: There was 1 patient hospital death at 2 months after repair. One patient died late that was not cardiac related. Survival was 92% +/- 6% at 8 months and remained constant thereafter. Four patients underwent reoperation (1 for residual aortic arch obstruction and 3 for subvalvular and valvular pulmonary stenosis). Freedom from reoperation decreased to stabilize at 83% +/- 8% after 2 years. The risk to have right ventricular outflow tract obstruction develop was 33% +/- 10% at 1 year, increasing slowly and leveling out at 57% +/- 12% at year 5 and thereafter. Statistical analysis revealed no significant risk factor for death or need for reoperation. CONCLUSIONS: The Taussig-Bing anomaly should be corrected in the neonatal period or in early infancy by arterial switch operation, closure of the ventricular septal defect, and simultaneous correction of associated cardiovascular anomalies as a one-stage procedure. Right ventricular outflow tract obstruction often complicates the postoperative course and is the main cause for reintervention.
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BACKGROUND: The global myocardial stress response during cardiac surgery has not been systematically studied, nor is it known whether the response of the neonatal myocardium is intrinsically different from that of older children. To determine the age-related molecular basis of this response, we conducted microarray-based differential gene expression profiling on right ventricular tissue samples acquired in patients of varying ages with right ventricular outflow tract obstruction. METHODS: We studied gene expression profiles in 24 patients during operations for lesions involving right ventricular outflow tract obstruction age stratified into group I (7 patients, aged 5 to 66 days; mean, 30 days) and group II (17 patients, aged 4 months to 12.5 years; mean, 2.8 years). Myocardial samples were taken from the right ventricular outflow tract after aortic occlusion and archived in liquid nitrogen. RNA isolation, fluorescence labeling of complementary DNA, hybridization to spotted arrays containing 19,008 characterized or unknown human complementary DNAs, and quantitative fluorescence scanning of gene-expression intensity were performed at the University of Toronto Health Network Microarray Centre. Data were analyzed with the Significance Analysis for Microarrays program. Minimum Information About Microarray Experiments-compliant, log2-normalized data sets were compared to ascertain potential statistical differences in gene expression between patient groups. RESULTS: There were no hospital deaths or major postoperative morbid events. We identified 50 transcripts differentially expressed in the neonatal group (the predicted false discovery rate was <0.8 transcripts). The neonatal pattern of gene expression (group I) was dominated by genes with literature-validated cardioprotective, antihypertrophic, and antiproliferative properties, including increases in atrial natriuretic peptide, protein phosphatase 2A, small GTPase rap1, and protein inhibitor of activated STAT protein, PIASy. Several transcripts have not been previously reported in heart. CONCLUSIONS: Neonatal myocardium has a unique pattern of gene expression, which may result from developmental (age-related) differences or reflect a more severe disease phenotype independent of age effects per se. The neonatal transcript profile seems to reflect a stress-induced protective program composed of genes with functions diametrically opposed to those expected to be related to the pathogenesis of critical right ventricular outflow tract obstruction, thus revealing a novel and compensatory antidisease transcriptional response in the neonatal heart.
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The clinical features and outcome after various surgical procedures on 9 patients with coarctation or interruption of the aortic arch and malalignment ventricular septal defect (group 1) were compared with those of 9 patients with the arch anomaly without malalignment ventricular septal defect (group 2). Cardiomegaly and metabolic acidosis were prominent in group 1. Five of the 9 patients in group 1 died in the immediate postoperative period (56% mortality), but no operative deaths occurred among 8 patients in group 2 (p less than 0.01). The ratio of left ventricular outflow tract to ascending aortic diameter was 0.59 +/- 0.09 in group 1 and 1.03 +/- 0.11 in group 2 (p less than 0.01). Three of 4 patients with a ratio of less than 0.6 died, but no operative deaths occurred among the 6 patients who had a palliative operation and in whom the ratio was more than 0.6. These data suggest that left ventricular outflow tract obstruction is critical when the ratio of left ventricular outflow tract to ascending aortic diameter is 0.6 or less. The presence of severe left ventricular outflow tract obstruction necessitates modification of the present surgical strategy.
After disappointing results with non-surgical measures there is now a move back towards surgical left ventricular outflow tract resection as a treatment of choice for hypertrophic obstructive cardiomyopathy. The traditional operation presents various technical difficulties and occasionally disappointing results. Using a diathermy loop to resect the hypertrophied outflow tract has provided a much more convenient and reliable technique. This paper describes an instrument incorporating all the features of this technique (Left ventricular resectoscope).
Aortic translocation is a useful surgical option in certain difficult subsets of transposition of great arteries with ventricular septal defect and left ventricular outflow tract obstruction. We report here the use of this technique with pulmonary homograft reconstruction of right ventricular pulmonary artery continuity in a child with transposition of the great arteries, left ventricular outflow tract obstruction, and restrictive ventricular septal defect.
Between 1975 and 1994, six patients under 15 years old with congenital aortic stenosis underwent relief of left ventricular outflow tract obstruction (LVOTO) by aortoseptal approach. Although we got satisfactory relief of LVOTO in all patients, there were two late deaths. In one patient with residual ventricular septal defect (VSD), intractable ventricular arrhythmia developed 12 years postoperatively. Although the residual VSD closure was performed, the patient died of the arrhythmia. The another one who was the youngest 4-year-old boy suddenly died 4 months postoperatively. The detail about his death was unknown, but we presumed ischemic heart attack as the cause of his death. In 3 of remaining survived 4 patients, we encountered a few complications. They were right ventricular outflow obstruction, coronary artery injury and conduction system injury. All complications and problems we encountered in this series were related to the incision and the reconstruction of aortoseptal approach. In conclusion, the aortoseptal approach is effective technique for relief of LVOTO, but on the other hand it is invasive and not so safe operation. So we should be cautious about these complications and problems.
Between August 1985 and May 1990, 27 neonates and infants underwent combined correction of intracardiac and aortic arch anomalies through a median sternotomy. Coarctation (CoA) was combined with VSD (6), AVSD (2), Taussig-Bing (TB) heart (5), transposition of the great arteries (TGA) (1), TGA + VSD (2), congenitally corrected TGA + VSD (1) and VSD + myxoid stenotic outlet valves (1). Interrupted aortic arch (IAA) was combined with VSD (10) and TB heart (1). Two patients had IAA type B as well as CoA. Age at operation varied from 2 to 243 days with a mean age of 51 days. Twenty patients (70%) were younger than 30 days. One TGA + VSD and all TB hearts had maligned outlet septum and right ventricular outflow tract obstruction (RVOTO). Posterior outlet septum deviation and left ventricular outflow tract obstruction (LVOTO) occurred in 8 patients with malalignment VSD and IAA (7) or CoA (1). Aortic arch reconstruction was performed using extended end-to-end anastomoses. In 3 patients, arch hypoplasia necessitated patch implantation. VSDs were closed through the right atrium unless the anatomy dictated otherwise. One TB heart was reconstructed with a Kawashima procedure. All other TB hearts and all TGAs were corrected with arterial switch operation. Obstructing outlet septum was resected whenever necessary. Follow-up was complete and included echo-Doppler control. Eleven patients had postoperative heart catheterisation. Early mortality was 18.5% (5 patients). Persisting LVOTO or RVOTO was responsible. There was no late mortality. Five patients were reoperated upon: 3 for stenotic anastomoses and 2 for a subaortic membranous stenosis. Successful balloon dilatation of recoarctation was performed four times.(ABSTRACT TRUNCATED AT 250 WORDS)
Valve conservation surgery represents an exciting advance in the evolution of valve surgery. Recent studies have shown the significant advantages of mitral valve repair over valve replacement. While there are significant advantages for valve repair, the surgeon requires a greater understanding of the mechanism of valvular dysfunction prior to repair and requires an accurate means to assess the adequacy of the repair in the operating room immediately following the repair. Intraoperative echocardiography with color flow Doppler mapping provides immediate and accurate assessment of cardiac anatomy, hemodynamics, and valve integrity. These data are vital for optimal intraoperative surgical decision making. Intraoperative echocardiography has an important role in the evaluation in patients undergoing surgery to the aortic valve and left ventricular outflow tract by the delineation of presence and mechanism of left ventricular outflow tract obstruction, the quantification of severity of the left ventricular outflow tract gradient, the severity and mechanism of aortic regurgitation, the distribution and severity of left ventricular hypertrophy, and identification of associated lesions such as mitral regurgitation. Aortic valve conservation surgery is more complex than mitral valve surgery. The surgical techniques for aortic valve repair have been slower to evolve than mitral repair with a much smaller percentage of patients currently suitable for valve repair. However, with the aid of intraoperative echocardiography, the future shows similar promise that has already been fulfilled with mitral valve repair. Even in its infancy, intraoperative echocardiography has become indispensable to the innovative cardiac surgeon. However, without consideration of adequate echocardiographic training, incorrect echocardiography diagnoses can lead to inappropriate surgical decisions.
UNLABELLED: The Ross procedure has been used in children and young adults for aortic valve replacement and the correction of complex obstruction syndromes of the left ventricular outflow tract. We report the mid-term results of the Ross procedure in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and December 2005, 18 patients were operated on using the Ross procedure. The mean age at the time of surgery was 12 years, being 12 patients male (67%). The primary indication for surgery was isolated aortic valve disease, being the predominant abnormality in 58% of cases aortic regurgitation and in 42% left ventricular outflow tract obstruction. Associated lesions included sub-aortic membrane in 3 patients (16%), small VSD in 2 patients (11%), bicuspid aortic valve in 4 patients (22%) and severe left ventricular dysfunction and mitral valve regurgitation in 1 patient (6%). Ten of the 18 patients (56%) had been submitted to previous surgical procedures or percutaneous interventions. RESULTS: Early post-operative mortality was not seen, but two patients (11%), had late deaths, one due to endocarditis, a year after the Ross procedure, and the other due to dilated cardiomiopathy and mitral regurgitation. The shortest time of follow-up is 6 months and the longest 72 months (median 38 months). Of the 16 survivors, 14 patients are in class I of the NYHA and 2 in class II, without significant residual lesions or need for re-intervention. The 12 patients with more than a year of follow up revealed normal coronary perfusion in all patients and no segmental wall motion abnormalities. Nevertheless, two of the 12 patients developed residual dynamic obstruction of LVOT and in three patients aortic regurgitation of a mild to moderate degree was evident. Significant gradients were not verified in the RVOT. CONCLUSIONS: The Ross procedure, despite its complexity, can be undertaken with excellent immediate results. Aspects such as the dilation of the neo aortic root and homograft evolution can not be considered in a study of this nature, seeing that the mean follow up time does not exceed 5 years.
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We report a 73-year-old woman with primary cardiac leiomyosarcoma in the left atrium and ventricle. The tumor progressed very rapidly for 2 months after initial clinical evaluation. Obstruction of the left ventricular outflow tract and mitral stenosis were induced by the tumor. Urgent surgical resection was performed because she had cardiogenic shock due to paroxysmal atrial fibrillation. We could not resect the tumor completely because of severe invasion. She refused postoperative chemotherapy and radiotherapy, and died suddenly at home 89 days after surgery. To our knowledge, this is the first observation of mitral stenosis concomitant with obstruction of the left ventricular outflow tract in a patient with primary cardiac leiomyosarcoma.
Two-dimensional Doppler echocardiography was used as an intraoperative cardiac function monitor in anesthetic management of a 79-year-old male with hypertrophic obstructive cardiomyopathy (HOCM) who underwent pulmonary lobectomy for lung cancer. Circulatory collapse occurred after thoracic epidural anesthesia (TEA), and was aggravated with following induction of general anesthesia. The collapse did not improve with phenylephrine nor atropine and necessitated ethylephrine and dopamine. During the above course, left ventricular outflow tract pressure gradient measured with continuous wave Doppler method was almost in proportion to cardiac output measured with thermo-dilution method. This means that TEA and the administration of inotropics did not worsen the left ventricular outflow tract obstruction. Left ventricular filling property estimated by trans-mitral flow velocity spectra improved when hemodynamics was stabilized with continuous infusion of dopamine, while it had been impaired during preoperative period and at the beginning of anesthesia. Our observation suggests that TEA for HOCM patient is a relative indication because it may exert negative inotropic effect, and that careful titration with inotropics is not contraindicated when undesired cardiac depression is proved by echocardiography.
A young child with hypertrophic obstructive cardiomyopathy, who was reoperated on for recurrent left ventricular outflow tract obstruction, underwent myectomy with patch augmentation of the ventricular septum. Cephalad resuspension of the aortic leaflet of the mitral valve resulted in abolishment of residual obstruction occurring secondary to its systolic anterior motion.
INTRODUCTION: Repaired tetralogy of Fallot is usually conducive to a normal and unrestricted lifestyle. However, occasionally, late sudden death occurs due to ventricular tachycardia. This is thought to be due to a combination of factors, including both left and right ventricular dilation, which may be associated with valvular incompetence and residual right ventricular outflow tract obstruction. Several studies have shown that QRS duration > 180 ms is a predictor for life-threatening ventricular arrhythmias. METHODS AND RESULTS: We recalled our tetralogy population (n = 57) to identify risk factors for prolonged QRS duration on the resting ECG. Factors examined included history, demographics, symptoms, surgery, chest X-ray, ECG and echocardiography. Extensive analysis found only a significant positive correlation between QRS duration and degree of residual right ventricular outflow tract obstruction. CONCLUSIONS: Our study suggests that residual right ventricular outflow tract gradients may be a very significant contributor to QRS prolongation. It will be interesting to see if future studies of larger cohorts confirm this finding.