Outflow Obstruction In Patients Research Articles

Features of left ventricular outflow obstruction in patients with different phenotypes of hypertrophic cardiomyopathy

Features of left ventricular outflow obstruction in patients with different phenotypes of hypertrophic cardiomyopathy

S531 Rates of Diagnosis of Ineffective Esophageal Manometry and Esophago-Gastric Junction Outflow Obstruction in Patients With and Without Hiatal Hernia Using Chicago 4.0 vs Chicago 3.0

S531 Rates of Diagnosis of Ineffective Esophageal Manometry and Esophago-Gastric Junction Outflow Obstruction in Patients With and Without Hiatal Hernia Using Chicago 4.0 vs Chicago 3.0

Transaortic Shallow Septal Myectomy and Cutting of Secondary Fibrotic Mitral Valve Chordae-A 5-Year Single-Center Experience in the Treatment of Hypertrophic Obstructive Cardiomyopathy.

Background: Anomalies of the mitral apparatus have been shown to contribute to left ventricular outflow obstruction in patients with hypertrophic cardiomyopathy (HCM). We report our 5-year single-center experience with a shallow myectomy procedure associated with transaortic mitral valve repair in a cohort of HCM patients. Methods: We studied 83 consecutive patients who underwent surgical treatment of symptomatic left ventricular outflow obstruction. In all study patients, a transaortic shallow septal myectomy was performed. Fibrous or muscular structures connecting the papillary muscles to the septum or free wall were resected, and fibrotic secondary chordae of the anterior mitral valve were cut selectively. Results: We report one death (1.2%) during hospitalization, no iatrogenic ventricular septal defects, and two (2.4%) mitral valve replacements. At discharge, no patients were in New York Heart Association (NYHA) Class III/IV, from 49 (59%) preoperatively. Mean maximal septal thickness decreased from 24 ± 6 to 16 ± 3 mm. Mean outflow gradient decreased from 93 ± 33 to 13 ± 11 mmHg. Grade 3 or 4 mitral regurgitation was noticed in one patient postoperatively, from 32 (39%) before surgery. Conclusions: Shallow septal myectomy associated with secondary mitral valve chordal cutting and papillary muscle mobilization provided excellent results offering adequate treatment of outflow obstruction.

Performance of Open and Closed Cell Laser Cut Nitinol Stents for the Treatment of Chronic Iliofemoral Venous Outflow Obstruction in Patients Treated at a Single Centre

A number of dedicated self expanding nitinol stents have been developed for use in the venous system, with both open cell (OC) and closed cell (CC) designs available. Data comparing these different designs are lacking. The objective of this study was to evaluate outcomes in patients treated with open and closed cells for unilateral chronic iliac vein obstruction. A single centre retrospective cohort study was conducted, including all patients treated with a dedicated nitinol venous stent between 2014 and 2019. Stent patency and details of re-interventions (including lysis, venoplasty, reinforcement, extension, arteriovenous fistula formation) were examined in the first post-operative year. Subgroup analysis described outcomes for patients treated with OC and CC stents ending above the inguinal ligament and those who required extension into the common femoral vein. Cox regression analysis was used to identify factors associated with loss of primary patency. A total of 207 patients were included (OC 100 patients, CC 107 patients). There was no significant difference between the groups for age (OC 42 years, CC 44 years); gender (OC and CC 67% female); presence of post-thrombotic lesions (OC 71%, CC 73%); stenting across the inguinal ligament (OC 58%, CC 56%), or presence of inflow disease (OC 49%, CC 47%). Primary and cumulative patency at 12 months were similar between groups (primary: OC 63%, CC 65%; cumulative: OC 93%, CC 90%). Patients with a CC stent across the inguinal ligament had a greater risk of needing multiple re-interventions at one year compared with those with an OC stent (odds ratio 2.84, 95% confidence interval [CI] 1.16 – 6.9) but overall, the only factor significantly associated with loss of primary patency was inflow vessel disease (hazard ratio 3.39, 95% CI 1.73 – 6.62, p < .001). OC and CC dedicated nitinol venous stents were observed to perform similarly in terms of patency and symptom improvement at one year. Disease of the inflow vessels was the most important factor associated with a loss of stent patency irrespective of stent design.

Performance of Open and Closed Cell Laser Cut Nitinol Stents for the Treatment of Chronic Iliofemoral Venous Outflow Obstruction in Patients Treated at a Single Centre

A number of dedicated self expanding nitinol stents have been developed for use in the venous system, with both open cell (OC) and closed cell (CC) designs available. Data comparing these different designs are lacking. The objective of this study was to evaluate outcomes in patients treated with open and closed cells for unilateral chronic iliac vein obstruction. A single centre retrospective cohort study was conducted, including all patients treated with a dedicated nitinol venous stent between 2014 and 2019. Stent patency and details of re-interventions (including lysis, venoplasty, reinforcement, extension, arteriovenous fistula formation) were examined in the first post-operative year. Subgroup analysis described outcomes for patients treated with OC and CC stents ending above the inguinal ligament and those who required extension into the common femoral vein. Cox regression analysis was used to identify factors associated with loss of primary patency. A total of 207 patients were included (OC 100 patients, CC 107 patients). There was no significant difference between the groups for age (OC 42 years, CC 44 years); gender (OC and CC 67% female); presence of post-thrombotic lesions (OC 71%, CC 73%); stenting across the inguinal ligament (OC 58%, CC 56%), or presence of inflow disease (OC 49%, CC 47%). Primary and cumulative patency at 12 months were similar between groups (primary: OC 63%, CC 65%; cumulative: OC 93%, CC 90%). Patients with a CC stent across the inguinal ligament had a greater risk of needing multiple re-interventions at one year compared with those with an OC stent (odds ratio 2.84, 95% confidence interval [CI] 1.16 - 6.9) but overall, the only factor significantly associated with loss of primary patency was inflow vessel disease (hazard ratio 3.39, 95% CI 1.73 - 6.62, p < .001). OC and CC dedicated nitinol venous stents were observed to perform similarly in terms of patency and symptom improvement at one year. Disease of the inflow vessels was the most important factor associated with a loss of stent patency irrespective of stent design.

Incidence and management of the left ventricular outflow obstruction in patients with atrioventricular septal defects.

OBJECTIVESLeft ventricular outflow tract obstruction (LVOTO) is a recognized complication after complete repair of atrioventricular septal defect (AVSD). This study reviewed the incidence and management of LVOTO following AVSD repair at a single institution.METHODSFrom 1975 to 2019, 24 patients (3.3%, 24/730) underwent reoperation due to LVOTO following partial AVSD (pAVSD) and complete AVSD (cAVSD) repair. The data were retrospectively reviewed.RESULTSThe incidence of LVOTO following pAVSD and cAVSD repair was 4.4% (12/275) and 2.6% (12/455). Freedom from LVOTO reoperation following pAVSD and cAVSD repair at 25 years was 94.3% [95% confidence interval (CI); 89.7–96.7] and 95% (95% CI; 91.1–97.3). The median time from complete repair of pAVSD and cAVSD to LVOTO reoperation was 4.4 years [interquartile range (IQR): 3.4–6.7] and 2.6 years (IQR: 2.2–4.7). Freedom from second LVOTO reoperation at 5, 10 and 15 years was 83.7% (95% CI; 57.2–98.2), 59.2% (95% CI; 28.7, 80.3) and 39.5% (95% CI; 13.2–65.3). The median time between the first and the second LVOTO reoperation in the groups of pAVSD and cAVSD was 6.1 years (IQR: 3.4–8.9) and 8.6 years (IQR: 5.7–9.8). There was no significant difference regarding the first (P = 0.7406) and subsequent LVOTO (P = 0.7153) following complete repair of pAVSD and cAVSD. Combined access to the left ventricular outflow tract was not protective regarding LVOTO reoccurrence. Survival for both groups after LVOTO reoperation at 15 years was 95.6% (95% CI 99.4–72.9).CONCLUSIONSIncidence of LVOTO after AVSD repair is low but the reoccurrence rate is high. Standard subaortic resection does not always provide definitive LVOTO relief. The survival after LVOTO reoperation is excellent.

Effects of combined myectomy with mitral valve repair on a three-dimensional mitral valve model and left ventricle outflow obstruction in patients with hypertrophic cardiomyopathy

Aim . To assess the effects of combined myectomy with mitral valve repair on a three-dimensional model of the mitral valve in patients with obstructive hypertrophic cardiomyopathy. Methods. 24 patients with obstructive hypertrophic cardiomyopathy and left ventricular outflow obstruction over 50 mm Hg at rest were recruited in a study. Eight patients underwent combined myectomy with mitral valve repair according to the Carpentier method. Seven patients underwent the Alfieri's edge-to-edge repair, and nine patients underwent secondary chordae resection using the Ferrazzi’s technique. Before combined myectomy and two weeks after it, all patients underwent standard transthoracic echocardiography and real-time 3D transesophageal imaging of the mitral valve, followed by quantitative 3D reconstruction of the mitral valve and calculation of the annulus, the leaflets, and the aorto-mitral angle. Results. Despite the selected mitral valve repair technique, we observed a decrease in the LVOT obstruction gradient. There were no differences in the residual obstruction gradient between the selected mitral valve repair technique. Patients with obstructive hypertrophic cardiomyopathy, who underwent combined myectomy and posterior mitral leaflet plasty valve according to the Carpentier approach, reported a correlation of a decrease in the LVOT obstruction with the non-planar angle (r = -0.83; p = 0.040), sphericity index (r = 0.83; p = 0.04) and a decrease in the velocity excursion of the annulus (r = 0.94; p = 0.005). Patients who underwent the Alfieri's edge-to-edge repair demonstrated that the residual LVOT obstruction gradient depended on the annulus height (r = 0.90; p = 0.04) and the ratio of this height to the commissural diameter of the annulus ( r = 0.90; p = 0.04). After secondary chordae resection, a decrease in the LVOT obstruction gradient correlated with the sphericity index (r = 0.77; p = 0.03), the anterolateral-posteromedial annulus diameter (r = -0.72; p = 0.04), the anterior (r = -0.78; p = 0.02) and posterior (r = -0.78; p = 0.02) leaflets, the ratio of the total leaflet length to the anteroposterior diameter of the annulus (r = -0.83; p = 0.01), the area (r = -0.76; p = 0.0з) and the mitral valve tenting height (r = -0.95; p = 0.00). Conclusion .Combined myectomy with mitral valve repair is the method of choice in the treatment of patients with obstructive hypertrophic cardiomyopathy. The comparison of three mitral valve repair techniques did not reveal any differences in the residual LVOT obstruction gradient. However, the Alfieri's edge-to-edge repair may be considered as the most physiological technique to repair dynamic LVOT obstruction.

Iliocaval outflow obstruction in patients with venous ulcers in a small comparison study between patients with primary varicose veins and chronic deep vein disease

ObjectiveIliocaval outflow obstruction was investigated in patients with venous ulcers caused by primary superficial disease and chronic deep vein disease MethodsAfter clinical assessment, patients with healed or active venous leg ulcers underwent lower extremity duplex ultrasound examination to identify the presence of venous disease in the superficial, deep and perforating systems. Bilateral contrast venography and intravascular ultrasound examination were then performed to determine the presence and degree of iliocaval obstructive lesions. ResultsThis retrospective study included a total of 59 patients with 71 legs presenting active or healed ulcer. There were 16 limbs (22.5%) with superficial venous reflux associated with normal infrainguinal deep veins (group I) and 55 limbs (77.5%) with infrainguinal post-thrombotic deep venous disease (group II). Using venography and intravascular ultrasound examination, the incidence of >50% of venous obstruction in the iliocaval system in groups I and II were 75% and 83.6%, respectively. All obstructive lesions in group I were nonthrombotic. However, group II included thrombotic, nonthrombotic and combinations of the two types of obstructions. Group II had more ulcers (1.73 ± 1.3 vs 1.17 ± 0.5; P = .03), larger ulcers (>6 cm in 34.1% vs 8.3%), longer duration of ulcers (71.3 ± 110.5 months vs 37.9 ± 40.4 months; P = .03), smaller diameter of refluxing superficial veins (7.7 ± 2.6 mm vs 15.1 ± 6.6 mm; P = .001), and higher incidence of occluded iliocaval systems (18 [32.7%] vs 0; P = .003) than group I. After the exclusion of bilateral cases and thrombotic obstructions, the incidence of >50% nonthrombotic obstruction in ipsilateral and contralateral sides was 76.5% and 24.4%, respectively (P = .003). ConclusionsThe results of this study revealed that the majority of patients with venous ulcers with either infrainguinal primary superficial or post-thrombotic deep venous disease had an element of iliocaval venous obstruction.

Surgical and endovascular central venous reconstruction combined with thoracic outlet decompression in highly symptomatic patients

BackgroundSubclavian vein stenosis or occlusion at the thoracic outlet is a problem associated with certain anatomic and environmental stresses (venous thoracic outlet syndrome [VTOS]), the presence of central venous catheters, and the high flows associated with arteriovenous (AV) access in the limb. We describe our experience with open and endovascular techniques for restoring patency in highly symptomatic patients. MethodsA prospectively collected database of patients was queried for patients treated for central venous obstructive disease in the setting of highly symptomatic VTOS and ipsilateral AV access from October 2011 to August 2016. ResultsDuring the study period, 54 procedures were performed in 53 patients (68% male; mean age, 50.1 years). Indications for operation were venous outflow obstruction in patients with conventional VTOS (n = 19) or costoclavicular junction stenosis associated with ipsilateral dialysis access (n = 34). All patients had significant symptoms of swelling or pain. Eight patients underwent on-table pharmacomechanical thrombolysis for acute occlusion. All patients underwent costoclavicular junction decompression, 48 by infraclavicular first rib resection and 5 by claviculectomy; 6 patients underwent sternoclavicular rotation (Molina procedure) in addition to rib resection for further exposure. Surgical reconstruction of the vein was employed in 18 patients (33%); 9 underwent interposition grafting, 1 had jugular turndown, and 8 had patch angioplasty. The one patient undergoing two procedures suffered acute occlusion after patch repair followed by jugular turndown. Four patients underwent surgical reconstruction after thrombolysis. Endovascular procedures were performed in 36 patients (67%); 23 underwent venous angioplasty alone, and 13, all with hemodialysis access-associated stenosis, underwent stenting. Mean operative time was 135 (±63.5) minutes, and mean estimated blood loss was 238 (±261) mL. Median length of stay was 4 days. Perioperative complications were noted in 14 (26.4%) patients, including wound complications (n = 6), cardiac complications (n = 4), reocclusion (n = 3), and hemothorax requiring chest tube placement (n = 1) in a patient undergoing on-table thrombolysis. Mean follow-up was 13.6 (0.6-58.5) months. Initial clinical symptom relief was experienced in 100% of patients at the time of hospital discharge. During follow-up, 5 (9.4%) patients developed recurrent symptoms, 6 (11.3%) had reocclusion of the central system, and 16 (30.2%) required reintervention for restenosis, all but 2 in patients with ipsilateral hemodialysis access. Mean time to reintervention was 134 (±285) days. ConclusionsGiven our decision-making threshold, both open and endovascular procedures are associated with relatively low morbidity and high efficacy for treatment of central venous occlusion in both symptomatic VTOS and AV access-associated subclavian vein disease. Restenosis is common in patients with a patent ipsilateral hemodialysis access.

Cardiac Ventricular Contractile Responses to Chronically Increased Afterload Secondary to Right Ventricular Outflow Obstruction in Patients With Tetralogy of Fallot

We examined the adaptive mechanism of the pulmonary ventricle (PV) in response to increased afterload secondary to pulmonary stenosis in tetralogy of Fallot (TOF, n = 47) and congenitally corrected transposition of the great arteries (cCTGA, n = 18), where the PV is morphologically different. We also elucidated the effects of such adaptation on systemic ventricular (SV) function. PV contractility, assessed by dp/dtmax, showed significant positive correlations with PV pressure (r = 0.82, p <0.01 for TOF and r = 0.78, p <0.01 for cCTGA) and pulmonary-to-systemic ventricular pressure ratio (r = 0.70, p <0.01 for TOF and r = 0.76, p <0.01 for cCTGA) in patients with both TOF and cCTGA. Notably, the slopes of these correlations were significantly higher in cCTGA than in TOF (p <0.01), suggesting enhanced contractile responses in cCTGA. Moreover, SV dp/dtmax showed significant positive correlations with PV dp/dtmax in patients with both TOF and cCTGA (r = 0.67, p <0.01 and r = 0.61, p <0.01, respectively), indicating positive ventricular-ventricular interaction. In this relationship, the slopes of correlations were significantly higher in TOF than in cCTGA (p = 0.024). These results, indicating different behaviors of PV contractile physiology and its interaction with the SV, may have important therapeutic implications when considering medical, catheter, and surgical interventions for pulmonary stenosis in these diseases. The results may also offer the potential for a new approach for improvement of prognosis, especially in cCTGA.

Total Correction of Tetralogy of Fallot: Do We Still Need Trans annular Patch

Objective To evaluate early results after surgical repair for tetralogy of Fallot; to find out the trends of repair technique and outcomes at NICVD. Study design Descriptive case series. Place & Duration of study Department of Paediatric Cardiac Surgery, National Institute of Cardiovascular Diseases Karachi, from January 2016 to December 2016. Methodology All patients operated for total correction of tetralogy of Fallot were included in the study. Each patient was followed for three months post repair and echocardiography was used for final assessment of gradient across right ventricular outflow tract. Results A total of 74 patients who underwent surgical repair of tetralogy of Fallot were included in the study. There were 45 (61%) males and 29 (39%) females with an age range from 2 year to 13 year. Mean age was 6.3 year. Repair was done through ventriculotomy with trans annular patch (TAP) in seven patients. Sixty-seven patients were operated without ventriculotomy. Patients in whom TAP was used had more severe pulmonary regurgitation, delayed extubation and long ICU stay than patients where repair was performed without ventriculotomy. Follow up echocardiography showed residual right ventricular outflow obstruction in patients operated without ventriculotomy and trans annular patch placement with mild obstruction in 29 (43%) patients while moderate and severe right ventricular outflow tract obstruction found in 36 (56%) and 2 (3%) patients respectively. All patients who underwent trans annular patch placement had moderate right ventricular outflow tract obstruction. Conclusion Despite concern about residual right ventricular outflow tract obstruction after correction of tetralogy of Fallot, our data showed moderate obstruction is well tolerated postoperatively. Key words Tetralogy of Fallot, Trans annular patch, Echocardiography.

Commentary on “The Effect of Deep Venous Stenting on Healing of Lower Limb Venous Ulcers”

It is now well accepted that placement of venous stents is the “method of choice” for treatment of pelvic venous outflow obstruction. George et al.1George R. Verma H. Ram B.L. Tripathi R.K. The effect of deep venous stenting on healing of lower limb venous ulcers.Eur J Vasc Endovasc Surg. 2014; x (xx–xx)Google Scholar performed computed tomography (CT) venography in patients with persistent venous leg ulcers after failed conservative treatment and superficial venous interventions. The authors proceeded with transfemoral/ascending phlebography when CT-venography findings suggested obstruction. Obstruction was found in 38 patients (44 limbs) and stents were placed. The actual number of patients tested to find these patients is not given. The endpoint was healing of the ulcer. The cumulative freedom from ulcer after stenting was at 2 years approximately 60%. This was achieved despite the presence of perforator insufficiency, deep reflux, and significant saphenous incompetence in 90%, 26%, and 10%, respectively, of patients with pre-intervention duplex scanning. Patients with healed ulcers continued to stay healed at a cumulative rate of approximately 83% at 2 years. This findings are line with the results of others as referred to in the study and point to the importance of iliofemoral venous outflow obstruction in the pathophysiology of venous ulcer formation. The prevalence of outflow obstruction in patients with recalcitrant venous ulcers is still unknown. It may be hard to decide since non-thrombotic iliac vein lesions are ubiquitous and we still don't know at what degree a venous stenosis is hemodynamically significant. I agree with the authors that it certainly appears to be worthwhile to search for an iliofemoral outflow obstruction in patients with leg ulcers, which are difficult to heal or recur with other treatment. The authors don't inform us what they considered a significant obstruction. In absence of lack of hemodynamic information, most would consider to treat an obstruction with a minimal diameter stenosis of >50% based on acceptable clinical outcome in patients stented with this criterion. Although very helpful in diagnosis and accurate placing of stents, intravascular ultrasound is unfortunately often too expensive to use. The authors used a so-called “trial angioplasty” or “diagnostic ballooning” when no obvious obstruction was seen. However, there is no information in the study what degree of “waisting” resulted in an intervention. The method has not been validated. I would caution those that use this technique. Large diameter balloons (especially if high pressure balloons are used) placed in the angle at the iliac confluence or at the internal iliac venous inflow may produce a false waist with the unfolding of the balloon. An accurate hemodynamic test for outflow obstruction, preferably non-invasive, needs to be developed to establish a defined indication for stenting. Until then awareness of the importance of obstruction, clinical signs and symptoms, and morphological criteria will have to do. A reduction of the ulcer size is not in itself an accepted endpoint, but could be used within a Venous Clinical Severity Score. A proper CEAP classification of the study patients was not given. It is difficult to analyze the reasons for lack of healing and recurrence of ulcers in this small amount of material. None was found. The presence of axial deep reflux, development of “new” reflux, occlusion of the stent system, recurrence of deep vein thrombosis, incompliance with concomitant compression therapy, etc., have certainly been pointed out in larger studies by others. The Effect of Deep Venous Stenting on Healing of Lower Limb Venous UlcersEuropean Journal of Vascular and Endovascular SurgeryVol. 48Issue 3PreviewTo report the outcomes of endovascular interventions on deep veins in patients with venous ulcers (C6). Full-Text PDF Open ArchiveRe. “The Effect of Deep Venous Stenting on Healing of Lower Limb Venous Ulcers”European Journal of Vascular and Endovascular SurgeryVol. 48Issue 6PreviewWe read with interest Peter Neglen's commentary on our paper.1,2 Full-Text PDF Open Archive

Chronic Cerebrospinal Venous Insufficiency and Cerebrospinal Fluid: Another Piece of the Puzzle?

Chronic cerebrospinal venous insufficiency (CCSVI) is a theory that has attempted to link venous outflow obstruction from the central nervous system (CNS) with neurologic abnormalities such as multiple sclerosis (MS). In some ways, it has been unfortunate that CCSVI was initially described in the context of a disease such as MS. Enthusiastic and optimistic patients and physicians embraced the early findings of Zamboni et al (1) as an ‘‘explanation’’ and ‘‘cure’’ for this debilitating condition. Venous disease was identified on venography and treated with angioplasty, leading to patient-reported symptomatic improvement. Understandably, this type of fundamental paradigm shift regarding an important disease such as MS was unsettling to many, and some neurologists countered these claims with a very reasonable demand for evidence. This led to additional studies that largely centered on the noninvasive diagnosis of CCSVI and the inconsistency of Doppler ultrasound (US) in identifying CCSVI in patients with MS (2–4). Unfortunately, the deep investment in current thinking regarding an autoimmune basis of MS led these investigators to conclude that, if a noninvasive imaging test cannot diagnose CCSVI, then CCSVI must not exist. In my opinion, these conclusions about noninvasive imaging should have led to a call for additional study and not the condemnation of an entire theory. Instead, this is exactly what happened, and the effect was to significantly dampen the enthusiasm surrounding CCSVI. So let’s back up. This phenomenon started with an observation made by Zamboni et al (5) of a venous outflow obstruction in patients with MS. On the basis of that observation, angioplasty and stent placement have been

Percutaneous transluminal venoplasty after venous pressure measurement in patients with hepatic venous outflow obstruction after living donor liver transplantation

The aim of this study was to evaluate retrospectively the outcome of percutaneous transluminal venoplasty (PTV) after venous pressure measurement in patients with hepatic venous outflow obstruction following living donor liver transplantation (LDLT). We studied 24 consecutive patients suspected of having hepatic venous outflow obstruction after LDLT. Pressure gradients were measured proximal and distal to the lesion, and gradient values >3 mmHg were considered hemodynamically significant. We evaluated the technical success, complications, outcome of venoplasty and recurrence, and the patency rate. In all, 11 female patients manifested a pressure gradient >3 mmHg across the anastomotic site; they underwent subsequent PVT. The initial balloon venoplasty procedure was technically successful in 10 of the 11 patients (91%), and the pressure gradient was reduced from 5.8 to 1.1 mmHg (P < 0.01). Clinical improvement was observed in 9 of these 10 patients; one patient failed to improve and underwent retransplantation. Recurrent obstruction occurred in four patients; they underwent PTV with (n = 2) or without (n = 2) stent placement. There were no major procedural complications. PTV following venous pressure measurement is an effective and safe treatment for venous outflow obstruction in patients subjected to LDLT. In patients with recurrent obstruction, re-venoplasty is recommended.

Whole-heart magnetic resonance imaging of isolated subpulmonary stenosis associated with hypertrophic cardiomyopathy

Whole-heart magnetic resonance imaging (MRI) was performed in a 59-year-old man who was referred to the department of cardiology because of effort dyspnea. Echocardiography revealed asymmetric hypertrophy of the interventricular septum (Figure 1; upper left panel). There was an obstruction of the right ventricular outflow tract during systole (Figure 1; arrow in upper right panel). Continuous wave Doppler (Figure 1; lower panel) revealed a maximum velocity of 3.0 m/s, which corresponded to a pressure gradient of 36 mmHg within the right ventricular outflow tract. A whole-heart MRI from the short-axis view showed asymmetric hypertrophy of the interventricular septum, which protruded into the right ventricular outflow tract (Figure 2; arrow). Cine MRI also showed asymmetric hypertrophy of the inter-ventricular septum, which obstructed the right ventricular outflow tract during systole (Figure 3; arrows). Figure 1 Figure 2 Figure 3 Isolated subvalvular pulmonary stenosis (ISPS) – subvalvular pulmonary stenosis without a ventricular septal defect – is a rare disorder that has been shown to be associated with right ventricular outflow obstruction in patients with hypertrophic cardiomyopathy (1,2). It is usually accompanied by left ventricular outflow obstruction, but isolated right ventricular obstruction occurs infrequently. In contrast to conventional MRI, which requires prospective projection settings for constructing the multiple cross-sectional images, whole-heart MRI can retrospectively depict arbitrary cross-sectional images from the previously acquired three-dimensional data. In addition, whole-heart MRI allows for evaluation of the anomalous coronary artery that often accompanies ISPS (3). Thus, whole-heart MRI has the potential to become a routine diagnostic modality in patients with suspected ISPS.

Hepatic venous outflow obstruction after hydatid cyst surgery: evaluation with contrast enhanced magnetic resonance angiography

The aim of this study is to present the contrast enhanced magnetic resonance angiography findings of hepatic venous outflow obstruction in patients in whom surgery had been performed for hepatic hydatidosis. No patient history of parasitic hepatic venous invasion or earlier hepatic venous outflow obstruction is present. Four men and three women with a history of hydatid cyst surgery underwent contrast enhanced magnetic resonance angiography between April 2001 and June 2006. The mean age was 37.7 years. The mean time duration between the last date of surgery and the date of magnetic resonance angiography imaging was 65.5 months. The site of the hydatid cyst was the right lobe in two patients, the medial segment of the left lobe in two patients, the liver dome in two patients, and the conjunction of the right lobe anterior-left lobe medial segments in one patient. One patient had undergone total and one patient had undergone partial lobectomy, and cystectomy was performed in five patients. On magnetic resonance angiograms, nonvisualization or stenosis of the hepatic veins was detected in all cases. In one patient thrombosis and in another patient severe stenosis of the inferior vena cava were associated. The portal hilum was displaced anterosuperiorly in five patients. Intrahepatic collaterals were present in six patients and extrahepatic collaterals were seen in three. Associated thrombosis in the left portal vein was found in two patients. We conclude that patients with complicated hydatid cysts and who have had postoperative complications should be checked not only for recurrence or abscess formation, but also for vascular changes. magnetic resonance angiography is a useful alternative imaging technique and can provide useful information at one session within several minutes in patients who had undergone surgery for hydatid cyst of the liver.

MDCT venography in the evaluation of inferior vena cava in Budd-Chiari syndrome: a road less travelled

Indian J Gastroenterol 2009: 28(Jan–Feb):3–4 Budd-Chiari syndrome is characterized by hepatic venous outfl ow obstruction, with or without involvement of the inferior vena cava (IVC), and can be due either to venous thrombosis or to membranous occlusion, often leading to chronic liver disease. It is a well known clinical entity in the Far East, the Middle East, India and Africa. 1 The site, extent, and rapidity of hepatic venous occlusion can be highly variable. Information on these aspects of venous obstruction is important for planning appropriate treatment for patients with BCS. Thus, imaging of the hepatic veins and IVC plays a crucial role in the management of patients with BCS. Several non-invasive diagnostic imaging techniques like color Doppler (CD), multidetector computed tomography (MDCT) and, more recently, magnetic resonance imaging (MRI) have all been used in patients with BCS. Because of wide variations in clinical presentations and venous anatomy, and availability of a variety of imaging modalities, patients with BCS need an individualized imaging strategy to obtain a complete evaluation of venous abnormalities and deciding an appropriate treatment plan. Since the clinical condition of patients with BCS can deteriorate rapidly, an early correct diagnosis and evaluation is important. Ultrasonography combined with CD has a diagnostic sensitivity of more than 75% to diagnose venous obstruction. It is also cheap, quick, non-invasive and widely available. Because of these advantages, it is usually the fi rst line of investigation to diagnose hepatic venous outfl ow obstruction in patients with liver disease. 2,3 Normal hepatic veins exhibit characteristic triphasic Doppler signal when patent. Classic fi ndings that suggest involvement of hepatic vein(s) in BCS are non-visualization of the vein(s), replacement of the vein with a fi brous cord-like structure, presence of a thrombus in the vein lumen and venous stenosis at the confl uence of the hepatic vein with the IVC. Demonstration of a caudate lobe vein equal to or larger than 3 mm in diameter is a specifi c sign of BCS, provided the patient does not have heart failure. 4 However, CD evaluation of hepatic veins in a swollen liver in BCS is often diffi cult and inaccurate, because the compressed hepatic veins may not be visible even though these they may be patent. In addition, it is sometimes diffi cult to detect occlusion of the left and middle hepatic veins using CD as cardiac pulsations are in close proximity with the left lobe of the liver. 4 Thus, though CD appears to be a good screening tool in the initial

Systolic Heart Failure in a Patient With Hypertrophic Obstructive Cardiomyopathy

Systolic Heart Failure in a Patient With Hypertrophic Obstructive Cardiomyopathy

Clinical characteristics and outcomes of patients with hypertrophic cardiomyopathy with latent obstruction

The purpose of this article is to describe the demographic and clinical features of patients with hypertrophic cardiomyopathy (HCM) and latent obstruction, with an emphasis on identifying factors associated with disease progression and survival. The presence of a resting left ventricular outflow obstruction in patients with HCM has been well described and is associated with increased symptoms and adverse long-term outcomes. However, less is known about patients with latent obstruction. Four hundred fifteen patients with echocardiographic or catheterization findings of latent obstruction, defined as a left ventricular outflow pressure gradient <30 mm Hg at rest, which increases to > or =30 mm Hg with provocation, were identified and included in the study group. The mean age was 55.0 +/- 17.9, and 226 (54.6%) patients were male. There were 330 (79.5%) patients with New York Heart Association (NYHA) functional class I and II at baseline. Fifty-nine (17.9%) of these patients had symptom progression requiring septal reduction therapy. Eighty-five patients had severe symptoms (NYHA functional class III and IV) at baseline, and 23 (27.1%) underwent septal reduction. Overall survival at 1, 5, and 10 years was 98%, 91%, and 81%, respectively. Survival among patients after undergoing invasive relief of outflow obstruction was equivalent to the general US population. Latent obstruction in HCM is an important pathophysiologic entity and may cause heart failure symptoms. One-third of patients in this referral series required invasive therapy for relief of symptoms. The evaluation of HCM patients with resting outflow tract gradients <30 mm Hg must include provocative maneuvers to identify this substantial subset of patients, preferably by physiologic exercise.

Usefulness of Tissue Doppler Imaging for Demonstrating Altered Septal Contraction Sequence During Dual-Chamber Pacing in Obstructive Hypertrophic Cardiomyopathy

Dual-chamber pacing reduces left ventricular (LV) outflow obstruction in patients with obstructive hypertrophic cardiomyopathy (HC), the mechanism of which lies in pacing-induced paradoxic septal motion. This study was conducted to test the hypothesis that tissue Doppler imaging (TDI) could demonstrate changes in the septal contraction sequence during dual-chamber pacing in patients with HC. TDI was performed in 16 patients (5 women; mean age 63+/-11 years) who underwent dual-chamber pacing for 7.6+/-2.1 year. With and without pacing, the time to peak systolic myocardial velocity was measured from the basal, mid, and distal segments in the 4 different LV walls. Without pacing, there was almost no longitudinal segmental asynchrony. During pacing, however, marked longitudinal segmental asynchrony appeared, especially in the anteroseptal wall (from p=NS to p<0.01 by analysis of variance) and the ventricular septum (from p<0.05 to p<0.01), with the time to peak velocity extremely prolonged at the distal segments. This was associated with a modest but significant decrease in the LV pressure gradient (from 20+/-8 to 14+/-7 mm Hg, p<0.01). In patients with obstructive HC, altered septal contraction sequence accounts for the reduced LV outflow obstruction during dual-chamber pacing, which was clearly demonstrated by TDI.