Pitfalls in clinical recognition and a novel operative approach for hypertrophic cardiomyopathy with severe outflow obstruction due to anomalous papillary muscle

BACKGROUND: Ventricular septal myotomy/myectomy (Morrow procedure) is the standard surgical option for severely symptomatic patients with hypertrophic cardiomyopathy (HCM) and marked basal obstruction to left ventricular outflow due to mitral valve systolic anterior motion. In some patients, however, congenital malformations of the mitral apparatus may be responsible for outflow obstruction; the failure to recognize this morphology before operation could have adverse consequences. METHODS AND RESULTS: We recently evaluated 2 patients with obstructive HCM operated on at Mayo Medical Center in 1997 who demonstrated direct anomalous papillary muscle insertion into the anterior mitral leaflet, producing muscular midcavity obstruction. This anomaly is potentially identifiable with echocardiography by exaggerated anterior displacement of hypertrophied papillary muscles within the left ventricular cavity and the direct continuity between papillary muscle and anterior leaflet associated with a rigid motion pattern of the mitral apparatus. Echocardiographic diagnosis, however, was confused in both patients by the association of systolic anterior motion of the mitral valve, probably produced by freely mobile margins of the mitral leaflet unencumbered by papillary muscle insertion, and in 1 patient probably representing a second and more basal level of obstruction. Because outflow tract morphology was judged unsuitable for conventional myotomy/myectomy, a novel surgical strategy was designed to remove the outflow gradient in which an extensive myectomy trough (wider at its apical than basal extent) was created within the ventricular septum to papillary muscle level; also, in 1 patient, attachment of anterolateral papillary muscle with the lateral free wall was partially severed to increase mobility of the mitral apparatus. After surgery, both patients reported substantial relief of symptoms and improved exercise tolerance and also showed reduced or abolished basal outflow obstruction. CONCLUSIONS: In HCM, outflow obstruction due to anomalous papillary muscle insertion directly into anterior mitral leaflet is challenging to identify but should always be contemplated before operative intervention. This important (but often unsuspected) congenital malformation may require alternative surgical strategies to standard myotomy/myectomy, similar to those described here.     

Imaging and radiologic management of upper urinary tract infections

Most infections of the upper urinary tract respond promptly to antibiotic therapy and imaging is not necessary. Patients with urinary obstruction, diabetes, or immunocompromise are more likely to develop complicated infection, abscess, or have unusual organisms. Chronic granulomatous processes involving the kidney are usually related to recurrent bacterial infections. Again, stone disease or obstruction is often an underlying problem. In those patients who do not respond promptly to treatment or have a more complicated clinical picture, imaging can assess the severity and extent of disease. CT scan is the study of choice for diagnostic evaluation in these patients and directs percutaneous intervention when appropriate. Placement of drainage catheters is often curative but also may allow the patient to stabilize until surgical treatment is accomplished. One exception is the diagnosis of pyonephrosis, which may be accomplished more easily by ultrasound. In these cases, PCN placement is generally needed and is performed under fluoroscopic guidance. Ultimately, however, definitive surgical intervention often is needed to relieve the underlying obstruction.     

Tracheal obstruction in achalasia: a role for airway stenting?

We describe an 82-year-old woman who presented with acute tracheal obstruction secondary to advanced, asymptomatic achalasia. Conventional treatment of her achalasia failed to relieve recurrent episodes of airway obstruction requiring endotracheal intubation. Because she was not fit for an operation, a Gianturco endotracheal stent was placed bronchoscopically. She remains without respiratory or upper gastrointestinal symptoms 2 years later. Recent information regarding the pathophysiology and surgical treatment of this complication is reviewed.     

Chronic mesenteric ischemia

Thirty-four patients (twelve men, 22 women, mean age 53[16-71] years) with chronic mesenteric ischaemia were operated upon between 1979 and 1992. The most frequent symptom was loss of weight (50%) and postprandial pain (44%). The mean interval between onset of symptoms and diagnosis was 35 months. Angiography revealed disease of the coeliac trunk (CT) or the superior mesenteric artery (SMA) in 16 patients, of only the SMA in ten, and of only the CT in eight. Revascularisation was obtained with an autologous vein graft in 21 patients (on the TC in 12, AMS in nine), while transaortic endarterectomy was performed in 15 (on the CT in seven, the AMS in nine). There was one perioperative death. 20 patients were symptom-free 1-126 months after the operation, while five still had residual symptoms even though improved in three. Seven patients had a recurrence of symptoms, three immediately after operation and four after an initial symptom-free period. These results show that freedom from symptoms can be achieved even in advanced stages of chronic mesenteric ischaemia by reconstructive surgery of the intestinal and visceral arteries. However, residual symptoms that are possibly not of a vascular nature may persist after successful vascular reconstruction. Patients with recurrent obstruction may become symptom-free by repeat surgery even many years later.     

Congenital complete tracheal rings. Report of a case

An unusual congenital tracheal anomaly is described. Complete (closed) tracheal rings, occupying the entire length of the trachea, have seldom been reported. Most affected patients succumb to respiratory infections in early infancy. Diagnosis of this anomaly is difficult during life, but radiographic and endoscopic examinations may be revealing. There is no known definitive treatment, although the airway may be managed long enough for body growth to relieve the obstruction. This rare anomaly should be considered in those cases of intermittent respiratory obstruction that are not associated with other more common causes of stridor.     

Transurethral prostatic thermotherapy with microwaves (prostatron system) in bladder obstruction secondary to benign prostatic hypertrophy

Since April, 1992, 178 patients with symptomatic benign prostatic hyperplasia were treated by TUMT (363 treatments). Before entering the study, all patients had a Madsen symptom score of > 8, peak flow rate of < 15 ml/s, or average flow rate of < 10 ml/s, and post voiding residual urine of < 300 ml/s. The prostatic length was classified into group I < 50 mm (101 patients) and group II < 50 mm (77 patients). TUMT with the Prostatron device (Technomed) was performed in one, two or three session(s) of one or two hour(s) with analgosedation and on an outpatient basis. After treatment all patients were catheterized for 1-3 weeks; the morbidity rate was very low. Three and six months after treatment, the Madsen symptom score, peak flow rate, average flow rate and postvoiding residual urine improved to a high statistical significance in both groups. TUMT for benign prostatic outflow obstruction proved to be an effective treatment throughout the study period, with minimum morbidity. It must be emphasized that the degree of prostatic enlargement or the severity of the symptoms does not indicate clinical success or failure. However, the degree of bladder outflow obstruction and the quality of treatment achieved are very important: a) In patients with severe obstruction, TURP or open surgery continues to be the treatment that affords rapid relief of their symptoms. b) The clinical response to TUMT is dose-dependent; i.e., higher thermal dose, longer session (2 h) and the use of different catheters enhance the therapeutic efficacy.     

Total anomalous pulmonary venous connections and consideration of the Fontan or one-ventricle repair

There is now a considerable literature that babies with right atrial isomerism have a poor outcome. The reasons for this are complex and multifactorial, but may be related at least in part to intrinsically small and abnormal pulmonary veins. We reviewed a series of consecutive patients seen at a single institution and found that babies with right atrial isomerism, severe pulmonary outflow tract obstruction or atresia, and total anomalous obstructed pulmonary veins had a grim outlook, especially those requiring operation in the neonatal period. Others have reported a similarly concerning outcome.     

Extending the concept of the autograft for complete repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: a report of ten cases of a modified procedure

BACKGROUND: In most cases of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, a Lecompte procedure (réparation à l'étage ventriculaire) is possible without interposition of a conduit between the right ventricle and pulmonary artery. However, the anterior location of the pulmonary arteries after the Lecompte maneuver may be a potential cause for right ventricular outflow obstruction, which continues to be reported in 5% to 25% of cases. We have used a tubular segment of aortic autograft to connect the pulmonary artery, left in the orthotopic posterior position (without the Lecompte maneuver), to the right ventricle in 10 consecutive patients with transposition, ventricular septal defect, and left ventricular outflow tract obstruction. METHODS: Ten consecutive patients aged 2 months to 11 years (mean 32 months) have undergone a modified Lecompte operation. Eight had severe pulmonary stenosis, two had pulmonary atresia, and four had a restrictive ventricular septal defect at the time of the operation. Two had multiple ventricular septal defects. Seven had undergone one (n = 5) or two (n = 2) previous modified Blalock-Taussig shunts. All patients underwent a total correction with left ventricular-aortic intraventricular connection (four needed a ventricular septal defect enlargement), connection between the right ventricle and pulmonary arteries with a tubular segment of autograft aorta, without the Lecompte maneuver (anterior location of the bifurcation of the pulmonary arteries) on the right (n = 6) or the left (n = 4) of the aorta. No valvular device was used for the right ventricular outflow repair. RESULTS: No early or late deaths occurred. One patient with multiple ventricular septal defects needed an early (2 weeks) reoperation for a residual muscular ventricular septal defect. All patients are currently in New York Heart. Association class I, without medications, in sinus rhythm, at a mean follow-up of 30 months. Late results up to 3.6 years show no calcification on the chest roentgenogram, and at the most recent echocardiogram, right ventricular pressures were low (25 to 40 mm Hg, mean 33 mm Hg) and no significant gradient (over 10 mm Hg) was found between the right ventricle and pulmonary arteries. Left and right ventricular function was satisfactory. CONCLUSION: This modification of the Lecompte operation using a segment of autograft allows an excellent early and late result, with no danger of compression of anteriorly placed pulmonary arteries, no significant right ventricular outflow obstruction, and normal appearance of the tubular autograft. In view of laboratory and clinical evidence, normal growth of the autograft can be anticipated. It allows an elective correction of transposition, ventricular septal defect, and left ventricular outflow tract obstruction without a previous Blalock-Taussig shunt (three patients) and correction at a young age (three patients younger than 1 year).     

Right ventricular bypass for palliation of cardiac sarcoma

A 33-year-old woman with a nonresectable right ventricular sarcoma and pulmonary outflow tract obstruction underwent a right ventricular bypass operation for symptomatic relief. The patient had an uneventful recovery and was asymptomatic on discharge, without jugular plethora or hepatomegaly. We consider this procedure to be an excellent palliative treatment of malignant right ventricular obstructive symptoms for improving the patient's quality of life.     

Identification of patients at increased risk for prolonged urinary retention following radioactive seed implantation of the prostate

PURPOSE: Urinary retention is a frequently reported complication following radioactive seed implantation of the prostate. If retention is refractory, a post-implant transurethral prostatic resection may ultimately be required to relieve obstruction, leading to an increased risk of urinary incontinence. In this series the incidence of prolonged urinary retention was determined, and the effect of pretreatment and treatment related factors was analyzed to identify high risk patients. MATERIALS AND METHODS: A total of 251 patients with organ confined prostate carcinoma underwent transperineal prostate seed implantation. Of the patients 114 were implanted with 103palladium (103Pd) and 137 with 125iodine seeds. Of the patients who were implanted with 103Pd 90 received 3 months of neoadjuvant hormonal therapy. All patients had International Prostate Symptom Scores (I-PSS) recorded before implantation to assess the degree of urinary symptoms. In the patients receiving neoadjuvant hormones prostate volumes and I-PSS were recorded before initiation of hormone treatment and 3 months later at the time of implant. RESULTS: Urinary retention developed in 14 patients requiring catheterization for more than 48 hours. Median time to onset was 1 day after implant. Of these patients 6 ultimately required transurethral prostatic resection to relieve urinary obstruction. No patient had urinary incontinence following implantation or transurethral prostatic resection. Multivariate analysis revealed that pretreatment I-PSS, and combined treatment with hormonal therapy and 103Pd predicted for the development of retention. Patients with I-PSS 20 or greater had a 29% risk, I-PSS 10 to 19, 11% risk and I-PSS less than 10, 2% risk of retention. Neither patient age, clinical stage, prostate specific antigen, Gleason score, use of 125I nor prostate volume was significant. A subgroup analysis of patients receiving hormonal therapy and 103Pd revealed that those with persistent urinary symptoms (I-PSS 10 or greater) following 3 months of hormonal therapy had the greatest risk of prolonged retention (37%). CONCLUSIONS: The overall risk of prolonged urinary retention following prostate implantation was low in our series. Using the I-PSS questionnaire, high risk patients can be identified before treatment. Patients with significant pretreatment urinary symptoms or persistent urinary symptoms following 3 months of hormonal therapy and then implantation with 103Pd have the greatest risk.     

Late postoperative hemodynamic results and cineangiocardiographic findings after Mustard atrial baffle repair for transposition of the great arteries

Forty-nine patients with transposition of the great arteries who underwent a Mustard atrial baffle repair between 1964 and 1971 were assessed late postoperatively. There have been five late deaths: two related to baffle obstruction, two from noncardiac causes, and one sudden and unexpected. Hemodynamic data were available in 42 patients and autopsy in four. Obstruction of the lower venous channel was not encountered. Three patients had severe obstruction of the upper venous channel and in four there was mild restriction. Two patients had severe pulmonary venous obstruction resulting in late death; mild asymptomatic obstruction could not be excluded with certainty in six patients. Tricuspid incompetence was infrequently encountered in patients with an essentially intact ventricular septum. Left ventricular outflow tract obstruction was found in eight patients. In three it was present after satisfactory but incomplete surgical relief and in five it had not been recognized prior to operation. In only one of the latter patients was the obstruction important. Seventeen patients were operated on prior to one year of age. While baffle obstruction was confined almost entirely to these patients, the five youngest patients, aged one to nine weeks at operation, had adequate pulmonary and caval compartments at restudy two to three years later.     

Transurethral needle ablation of the prostate: a minimally invasive treatment for symptomatic benign prostatic hyperplasia

Transurethral needle ablation of the prostate, a relatively new minimally invasive treatment modality for patients with bladder outlet obstruction attributable to an enlarged prostate gland, has undergone extensive evaluation by numerous investigators worldwide. The results to date indicate that needle ablation is safe and effective for relieving symptoms in patients with benign prostatic hyperplasia, and the effect has been demonstrated to be durable for at least 2 years. Nevertheless, additional investigations with longer follow-up data are needed to address the important issues of extended durability (5 to 10 years) and biophysiologic mechanism of action. Comparisons between transurethral needle ablation of the prostate and transurethral resection of the prostate (TURP) have revealed that the subjective and objective measures of response are comparable, although TURP has consistently displayed a slight advantage over needle ablation for most variables analyzed, except quality of life score. The advantages of needle ablation over TURP are (1) performance in the office as an outpatient procedure, (2) no need for general or spinal anesthesia, (3) rapid recovery, (4) minimal side effects, and (5) one-time intervention. The following disadvantages exist with needle ablation: (1) it may not be indicated or effective in patients with large prostate glands (75 g or more); (2) no prostate tissue is available for histologic evaluation; and (3) no long-term efficacy or re-treatment rate data have been published. Overall, the available information indicates that transurethral needle ablation is a viable minimally invasive treatment that may be applicable in men with moderate to severe bladder outlet obstruction as a result of an enlarged prostate gland.     

Endoscopic Nd:YAG laser treatment of inoperable lower gastrointestinal cancer

Many patients with colorectal cancer are not amenable to curative resection at the time of presentation. Nevertheless, palliative resection still remains as the treatment of choice in the majority of patients. A small group of patients that are poor candidates for surgical resection may benefit from some non-surgical palliative procedures to relieve their symptoms. Electrocoagulation, cryosurgery and radiotherapy are some of the non-surgical procedure used and they are associated with high morbidity and mortality. The use of Neodymium: Yttrium-Aluminium-Garnet (Nd:YAG) laser photoablation to palliate patients with advanced colorectal carcinoma is well documented. It is associated with relatively low morbidity and perioperative mortality. It requires no anaesthesia and is the only non-surgical procedure that can be safely carried out above the peritoneal reflection. Nd:YAG laser had been used in some centres as a preresectional procedure in patient presenting with high grade obstruction. It allows proper bowel preparation followed by primary excision and anastomosis. As a palliative procedure, most patients showed rapid improvement in obstructive symptoms, bleeding and rectal discharge. The size of the lesion and circumferential extent of the tumour base correlate well with the response rate. Most patients remained asymptomatic before they succumb to the advanced disease. In our series, good palliation of obstructive symptoms was achieved in all obstructive cases with one laser treatment, bleeding tumours required an average of two sessions for complete haemostasis. In conclusion, Nd:YAG laser therapy is a safe and efficacious means for palliation of obstructive symptoms and bleeding in advanced rectal carcinoma.     

Initial Groningen experiences with dynamic cardiomyoplasty

Four patients, one woman and three men aged 48, 62, 49, en 54 years respectively, were subjected to cardiomyoplasty because of medically refractory heart failure secondary to ischaemic or idiopathic dilating cardiomyopathy. The operation and the training period were uncomplicated. In one patient symptoms of heart failure did not improve; the other patients experienced substantial relief of symptoms. However, all three died suddenly within one year after the operation, probably due to ventricular arrhythmias. Cardiomyoplasty may deserve a place in the treatment of heart failure, provided sudden death can be better prevented. Possibly, treatment with an implantable cardioverter-defibrillator might be useful.     

Cast syndrome: the superior mesenteric artery syndrome

Cast syndrome, clinically known as superior mesenteric artery syndrome (SMAS), is gastric dilatation with partial or complete obstruction of the duodenum. Although rare, it is most frequently seen in orthopaedic patients who have had spinal surgery or who are in hip spica or body casts. Obstruction occurs when there is compression of the duodenum between the superior mesenteric artery anteriorly and the aorta and spinal column posteriorly. Obstruction can occur within days of surgery or casting or may not develop for several weeks. Treatment for SMAS varies from conservative nonoperative to operative procedures. Complications can be severe if symptoms are not quickly recognized and treatment instituted in a timely manner.     

Gonorrhoea in the asymptomatic patient: presentation and the role of contact tracing for heterosexual men and women and for homosexual men

Clinically silent gonorrhoea is the major problem in the control of the disease. Only 12 per cent of infected women reported in 1974 because of symptoms, compared with 97 per cent of infected heterosexual men and only 35 per cent of homosexual men with gonococcal proctitis alone. Homosexual men, compared with heterosexual men, had twice as many subsequent sexual contacts after infection and had a higher incidence of early syphilis. Eighty-four per cent had experienced passive anorectal intercourse. Ninety-seven per cent of men with gonococcal urethritis reported because of symptoms, but occasionally (particularly after unsuccessful treatment) urethral gonorrhoea in men may be clinically silent and even require tests of the overnight urethral secretion for diagnosis. For women, and for homosexual men who have had passive anorectal (or oral) intercourse, the indication for attendance for tests for gonorrhoea should be having run the risk, and not the presence of symptoms. Routine tests of the anorectum for gonorrhoea are essential in cases of 80 women at risk, and for most homosexual men since over 80 per cent of these men will have had passive anorectal intercourse. Because gonococcal infections following treatment-failure are often clinically silent in both women and men, symptoms cannot be relied upon to indicate such failure. Follow-up smears and cultures are always essential.     

The indications for operations on the penile veins in venogenic erectile impotence

Unsatisfactory results of surgical management of venous erectile failure are attributed to difficult selection of relevant patients. The operation is to be performed in patients with isolated distal venous outflow. The diagnosis of occult proximal or mixed outflow may be made at dynamic cavernosography with loading test on the device for stimulation of erection. After obtaining the erection and removal of the constriction ring, distal venous outflow is blocked, thus creating the conditions for detection of occult proximal venous outflow. Surgical treatment (ligation of the dorsal vein or its insertion under tunica albuginea) is indicated only for patients free of mixed venous outflow.     

Dose-response relationship in radioiodine therapy of hyperthyroidism]

The course of HOCM is characterized by a slow progression. Symptoms can often be influenced favourably by medical treatment. Altogether there is some difference between the course of untreated and the propranolol-treated patient groups which is, however, not statistically significant. Furthermore, the rate of sudden death is uninfluenced. Comparing clinical and haemodynamic results in medicically treated patients we find a reduction of the outflow tract obstruction in case with clinical improvement but not a reduction of the enddiastolic pressure. Independent on the clinical course there was a significant increase of the enddiastolic pressure after 5.5 years. We may conclude that there is a progredient process of hypertrophy which is uninfluenced by the obstruction and by conservative management. The best results are obtained in the operated group. According to our results a surgical intervention should possibly be considereed more generously.     

Subaortic obstruction and the Fontan operation

Systemic outflow tract obstruction in the heart with a functional single ventricle promotes myocardial hypertrophy, and this has been shown to be an unequivocal risk factor for poor outcome at Fontan procedure. Such systemic outflow tract obstruction may be congenital or acquired. Those factors contributing to acquired systemic outflow tract obstruction in those patients with a double-inlet left ventricle, a rudimentary right ventricle, and a discordant ventriculoarterial connection include the size of the ventricular septal defect, previous pulmonary artery banding, and other volume unloading surgical procedures. Staging with a bidirectional cavopulmonary connection and construction of a proximal pulmonary artery-aortic connection or ventricular septal defect enlargement has neutralized this factor.     

The role of urodynamics in the evaluation of voiding dysfunction in men after cerebrovascular accident

PURPOSE: The etiology of voiding dysfunction was determined in men after a cerebrovascular accident who were at risk for obstructive uropathy to evaluate whether the cause of voiding dysfunction could be predicted by the type (obstructive or irritative) or onset of symptoms. MATERIALS AND METHODS: We evaluated 38 men with complaints of voiding dysfunction following a cerebrovascular accident. All patients were of the age when bladder outlet obstruction secondary to benign prostatic hyperplasia would otherwise be prevalent. After a comprehensive history and physical examination, all patients underwent multichannel urodynamic studies at a medium fill rate (20 to 50 ml. per minute). Findings were classified by the Abrams-Griffiths nomogram as obstruction, no obstruction or equivocal. RESULTS: Mean patient age was 70 years (range 54 to 87). Patients were grouped according to the presenting voiding complaints (purely irritative in 42%, purely obstructive in 34% or mixed in 24%). In 34 patients (89%) the onset of symptoms paralleled the occurrence of the cerebrovascular accident. Detrusor hyperreflexia was noted in 82% of the patients. There was no statistically significant difference in the occurrence of detrusor hyperreflexia among the 3 symptom groups (Fisher's exact test). Pressure-flow analysis clearly showed obstruction in 24 patients (63%), no obstruction in 9 (24%) and equivocal results in 5 (13%) according to the nomogram. There was no statistically significant difference in the incidence of obstruction among the 3 symptom groups (Fisher's exact test). CONCLUSIONS: Presenting symptoms did not predict the urodynamic findings of bladder outlet obstruction or detrusor hyperreflexia. The significant incidence of onset of symptoms after stroke suggests that the cerebrovascular accident induced voiding dysfunction in the face of preexisting bladder outlet obstruction may exacerbate the symptoms of the latter condition or vice versa.