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1.
Circ Arrhythm Electrophysiol ; 17(7): e012570, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39012930

ABSTRACT

BACKGROUND: Patients with refractory, symptomatic left ventricular (LV) mid-cavity obstructive (LVMCO) hypertrophic cardiomyopathy have few therapeutic options. Right ventricular pacing is associated with modest hemodynamic and symptomatic improvement, and LV pacing pilot data suggest therapeutic potential. We hypothesized that site-specific pacing would reduce LVMCO gradients and improve symptoms. METHODS: Patients with symptomatic-drug-refractory LVMCO were recruited for a randomized, blinded trial of personalized prescription of pacing (PPoP). Multiple LV and apical right ventricular pacing sites were assessed during an invasive hemodynamic study of multisite pacing. Patient-specific pacing-site and atrioventricular delays, defining PPoP, were selected on the basis of LVMCO gradient reduction and acceptable pacing parameters. Patients were randomized to 6 months of active PPoP or backup pacing in a crossover design. The primary outcome examined invasive gradient change with best-site pacing. Secondary outcomes assessed quality of life and exercise following randomization to PPoP. RESULTS: A total of 17 patients were recruited; 16 of whom met primary end points. Baseline New York Heart Association was 3±0.6, despite optimal medical therapy. Hemodynamic effects were assessed during pacing at the right ventricular apex and at a mean of 8 LV sites. The gradients in all 16 patients fell with pacing, with maximum gradient reduction achieved via LV pacing in 14 (88%) patients and right ventricular apex in 2. The mean baseline gradient of 80±29 mm Hg fell to 31±21 mm Hg with best-site pacing, a 60% reduction (P<0.0001). One cardiac vein perforation occurred in 1 case, and 15 subjects entered crossover; 2 withdrawals occurred during crossover. Of the 13 completing crossover, 9 (69%) chose active pacing in PPoP configuration as preferred setting. PPoP was associated with improved 6-minute walking test performance (328.5±99.9 versus 285.8±105.5 m; P=0.018); other outcome measures also indicated benefit with PPoP. CONCLUSIONS: In a randomized placebo-controlled trial, PPoP reduces obstruction and improves exercise performance in severely symptomatic patients with LVMCO. REGISTRATION: URL: https://clinicaltrials.gov/study; Unique Identifier: NCT03450252.


Subject(s)
Cardiac Pacing, Artificial , Cardiomyopathy, Hypertrophic , Cross-Over Studies , Ventricular Function, Left , Humans , Male , Female , Cardiac Pacing, Artificial/methods , Middle Aged , Cardiomyopathy, Hypertrophic/therapy , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/diagnosis , Treatment Outcome , Aged , Quality of Life , Time Factors , Hemodynamics , Ventricular Outflow Obstruction/physiopathology , Ventricular Outflow Obstruction/therapy , Ventricular Outflow Obstruction/diagnosis , Exercise Tolerance , Ventricular Function, Right , Recovery of Function
2.
Pediatr Cardiol ; 45(5): 1132-1141, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38480570

ABSTRACT

Cardiac rhabdomyomas are the most common benign pediatric heart tumor in infancy, which are commonly associated with tuberous sclerosis complex (TSC). Most rhabdomyomas are asymptomatic and spontaneously regress over time. However, some cases especially in neonates or small infants can present with hemodynamic instability. Surgical resection of the tumor, which has been the gold standard in alleviating obstruction, is not always possible and may be associated with significant morbidity and mortality. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be safe and effective in the treatment of TSC. We present the outcomes of neonates and an infant who received treatment for symptomatic rhabdomyomas at a tertiary cardiology center. Medical records were reviewed to obtain clinical, demographic, and outcome data. Six patients received interventions for symptomatic rhabdomyomas, median age at presentation was 1 day old (range from 1 to 121 days old), and 67% of the patients had a pathogenic mutation in TSC gene. One patient underwent surgical resection of solitary tumor at right ventricular outflow tract (RVOT) successfully. In the four patients with left ventricular outflow tract (LVOT) obstruction, two patients received combined therapy of surgical debulking of LVOT tumor, Stage I palliation procedure, and mTORi and two patients received mTORi therapy. One patient with RVOT obstruction underwent ductal stenting and received synergistic mTORi. Four of the five patients had good response to mTORi demonstrated by the rapid regression of rhabdomyoma size. 83% of patients are still alive at their latest follow-up, at two to eight years of age. One patient died on day 17 post-LVOT tumor resection and Hybrid stage one due to failure of hemostasis, in the background of familial factor VII deficiency. Treatment of symptomatic rhabdomyoma requires individualized treatment strategy based on the underlying pathophysiology, with involvement of multidisciplinary teams. mTORi is effective and safe in inducing rapid regression of rhabdomyomas. A standardized mTORi prescription and monitoring guide will ensure medication safety in neonates and infants with symptomatic cardiac rhabdomyoma. Although the majority of tumors responded to mTORi, some prove to be resistant. Further studies are warranted, ideally involving multiple international centers with a larger number of patients.


Subject(s)
Heart Neoplasms , Rhabdomyoma , Ventricular Outflow Obstruction , Humans , Heart Neoplasms/therapy , Heart Neoplasms/surgery , Heart Neoplasms/complications , Rhabdomyoma/complications , Rhabdomyoma/surgery , Rhabdomyoma/diagnosis , Rhabdomyoma/therapy , Infant , Infant, Newborn , Male , Female , Ventricular Outflow Obstruction/etiology , Ventricular Outflow Obstruction/therapy , Ventricular Outflow Obstruction/surgery , Retrospective Studies , Treatment Outcome , Echocardiography , Tuberous Sclerosis/complications , Tuberous Sclerosis/therapy , Tuberous Sclerosis/diagnosis , Cardiac Surgical Procedures/methods , MTOR Inhibitors/therapeutic use
3.
Can J Cardiol ; 40(5): 851-859, 2024 May.
Article in English | MEDLINE | ID: mdl-38122929

ABSTRACT

Hypertrophic cardiomyopathy is a common inherited cardiac condition where the myocardium progressively thickens in the absence of abnormal loading conditions. Left ventricular hypertrophy often leads to outflow tract obstruction, and this confers significant mortality and morbidity implications. Septal reduction therapies aim to relieve the obstruction in an attempt to reduce the burden of symptoms and potentially improve prognosis. However, both surgical and catheter-based approaches to septal reduction carry risks. At present, international guidelines and expert consensus statements suggest surgical myomectomy is the gold-standard treatment. In this point-counterpoint review, we discuss why in our opinion this recommendation should be reconsidered. We hope to cover the history of catheter based septal reduction therapies and the significant advances made over the last two decades. We also hope to show why we believe the current evidence shows catheter-based alcohol septal ablation is superior to surgical myomectomy.


Subject(s)
Cardiomyopathy, Hypertrophic , Catheter Ablation , Ventricular Outflow Obstruction , Humans , Cardiomyopathy, Hypertrophic/therapy , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnosis , Ventricular Outflow Obstruction/etiology , Ventricular Outflow Obstruction/diagnosis , Ventricular Outflow Obstruction/surgery , Ventricular Outflow Obstruction/therapy , Catheter Ablation/methods , Heart Septum/surgery
5.
BMC Cardiovasc Disord ; 21(1): 372, 2021 08 03.
Article in English | MEDLINE | ID: mdl-34344308

ABSTRACT

BACKGROUND: Complete atrioventricular block (AVB) is a life-threatening condition that usually occurs in elderly people with organic heart disease. We herein describe a rare case of complete AVB in a young man with hypertrophic obstructive cardiomyopathy (HOCM) complicated by cholecystitis and cholangitis. Both cardio-biliary reflex and alcohol septal ablation (ASA) can cause conduction block, but the latter is often irreversible. However, their simultaneous occurrence in a patient has not been reported. CASE PRESENTATION: A 31-year-old man presented with acute cholecystitis and cholangitis and complete AVB, which had been diagnosed at a local hospital on the third day after onset. On the fourth day, he was transferred to the emergency department of our hospital because of persistent complete AVB, although his abdominal pain had been partially relieved. An echocardiogram showed a remarkably elevated left ventricular outflow tract (LVOT) gradient (105.2 mmHg) despite the performance of ASA 9 years previously. The abdominal pain gradually disappeared, and normal sinus rhythm was completely recovered 11 days after onset. We determined that cardio-biliary reflex was the cause of the AVB because of the absence of other common causes. Finally, the patient underwent implantation of a permanent pacemaker to reduce the LVOT obstruction and avoid the risk of AVB recurrence. CONCLUSIONS: Cholecystitis is a rare cause of complete AVB, which is a difficult differential diagnosis when complicated by HOCM after ASA. Clinicians should be alert to the possibility of cholecystitis in patients with abdominal pain and an unknown cause of bradycardia, complete AVB, or even sinus arrest.


Subject(s)
Ablation Techniques/methods , Atrioventricular Block/etiology , Cardiomyopathy, Hypertrophic/complications , Cholecystitis/complications , Reflex , Acute Disease , Adult , Atrioventricular Block/diagnosis , Atrioventricular Block/therapy , Bundle-Branch Block/diagnosis , Cholangitis/complications , Cholecystitis/diagnostic imaging , Echocardiography , Electrocardiography , Ethanol/therapeutic use , Humans , Male , Ventricular Outflow Obstruction/diagnostic imaging , Ventricular Outflow Obstruction/therapy
6.
Am J Cardiol ; 150: 110-113, 2021 07 01.
Article in English | MEDLINE | ID: mdl-34011439

ABSTRACT

Fabry disease (FD) is an X-linked linked genetic disorder caused by α-galactosidase A deficiency. The typical clinical manifestation is left ventricular hypertrophy, often mimicking hypertrophic cardiomyopathy (HC). In contrast to sarcomeric HC, left ventricular outflow tract obstruction (LVOTO) is less frequent. We describe 6 male patients with genetically confirmed FD and symptomatic LVOTO. All of them underwent a transcatheter alcohol septal ablation with an immediate effect on the obstruction in all cases and without any serious complications. The median LVOT maximal pressure gradient was 85 (60 to 170) mm Hg. The hemodynamic effect persisted during subsequent follow-up (ranging from 6 months to 16 years). Five patients reported substantial symptomatic improvement. Four patients were receiving specific FD therapy before the interventional procedure. In conclusion, alcohol septal ablation appears to be effective in the treatment of LVOTO in patients with FD and appears to be comparable to the limited published experience with surgical septal myectomy. Despite some important differences between FD HC and sarcomeric HC, the recommendation for treating LVOTO should be similar.


Subject(s)
Ablation Techniques/methods , Ethanol/therapeutic use , Fabry Disease/complications , Ventricular Outflow Obstruction/etiology , Ventricular Outflow Obstruction/therapy , Adult , Echocardiography , Electrocardiography , Fabry Disease/drug therapy , Humans , Male , Middle Aged , Ventricular Outflow Obstruction/diagnostic imaging
7.
J Med Case Rep ; 15(1): 269, 2021 May 18.
Article in English | MEDLINE | ID: mdl-34001279

ABSTRACT

BACKGROUND: In cases of hypertrophic obstructive cardiomyopathy (HOCM), the systolic anterior motion of the mitral valve apparatus results in an obstruction of the left ventricular outflow tract (LVOT), which is known as the SAM [systolic anterior motion] phenomenon. Hypothetically, a pathological obstruction of the LVOT of a different etiology would result in a comparable hemodynamic instability, which would be refractory to inotrope therapy, and may be detectable through echocardiography. CASE PRESENTATION: We observed a severely impaired left ventricular function due to a combination of a thrombotic LVOT obstruction and distinctive mitral regurgitation in a 56-year-old Caucasian, female patient after massive transfusion with aggressive procoagulant therapy. Initially, the patient had to be resuscitated due to cardiac arrest after a long-distance flight. The resuscitation attempts in combination with lysis therapy due to suspected pulmonary artery embolism were initially successful but resulted in traumatic liver injury, hemorrhagic shock and subsequent acute respiratory distress syndrome (ARDS). Oxygenation was stabilized with veno-venous extracorporeal membrane oxygenation (ECMO), but the hemodynamic situation deteriorated further. Transesophageal echocardiography (TEE) showed a massive, dynamic LVOT obstruction. Two thrombi were attached to the anterior leaflet of the mitral valve, resulting in a predominantly systolic obstruction. Unfortunately, the patient died of multiple-organ failure despite another round of lysis therapy and escalation of the ECMO circuit to a veno-venoarterial cannulation for hemodynamic support. CONCLUSION: Massive transfusion with aggressive procoagulant therapy resulted in mitral valve leaflet thrombosis with dynamic, predominantly systolic LVOT obstruction, comparable to the SAM phenomenon. The pathology was only detectable with a TEE investigation.


Subject(s)
Cardiomyopathy, Hypertrophic , Mitral Valve Insufficiency , Shock, Hemorrhagic , Ventricular Outflow Obstruction , Female , Humans , Middle Aged , Mitral Valve/diagnostic imaging , Mitral Valve Insufficiency/diagnostic imaging , Shock, Hemorrhagic/etiology , Shock, Hemorrhagic/therapy , Ventricular Outflow Obstruction/diagnostic imaging , Ventricular Outflow Obstruction/etiology , Ventricular Outflow Obstruction/therapy
8.
Int Heart J ; 62(2): 329-336, 2021 Mar 30.
Article in English | MEDLINE | ID: mdl-33731518

ABSTRACT

The reasons of residual left ventricular outflow tract (LVOT) obstruction following alcohol septal ablation (ASA) remain unclear, and outcomes of myectomy following failed ASA remain underreported.Thirteen symptomatic patients (10 women, a median age of 60.0 years) who underwent septal myectomy following failed ASA were reviewed. The patients were followed up for a median of 6 months. The clinical characteristics and outcomes of these patients were analyzed and were compared with those of 178 patients who underwent isolated myectomy without previous ASA at our institution during the same period.In the first ASA procedure, the median number of septal perforator arteries injected was 1.0 with the median value of peak creatine kinase following ablation of 978.5 U/L.Uncontrollable extent and location of infarcted myocardium caused by ablation and mitral subvalvular anomalies were found in four (30.8%) and seven (53.8%) patients, respectively. No operative or follow-up deaths occurred. The median maximum LVOT gradients fell from preoperative 112.0 to 8.5 mmHg at follow-up (P < 0.001). Compared with controls, patients with failed ASA had a higher proportion of mitral subvalvular anomalies (53.8% versus 13.5%, P = 0.001) and developed a higher incidence of complete atrioventricular block following myectomy (15.4% versus 1.7%, P = 0.038).Low institutional or operator experience with ablation, uncontrollable extent and location of infarcted myocardium caused by ablation, and mitral subvalvular anomalies may be reasons for failed ASA. Surgical myectomy for the treatment of residual LVOT obstruction after unsuccessful ASA may be associated with favorable results.


Subject(s)
Ablation Techniques/adverse effects , Cardiac Surgical Procedures/methods , Ethanol/pharmacology , Heart Septum/surgery , Ventricular Outflow Obstruction/therapy , Adult , Aged , Female , Follow-Up Studies , Heart Rate/physiology , Heart Septum/diagnostic imaging , Humans , Male , Middle Aged , Retrospective Studies , Treatment Failure , Ventricular Outflow Obstruction/diagnosis , Ventricular Outflow Obstruction/physiopathology
9.
Catheter Cardiovasc Interv ; 97(1): 152-156, 2021 01 01.
Article in English | MEDLINE | ID: mdl-32812368

ABSTRACT

Owing to the growing population with end stage heart failure, the use of left ventricular assist devices (LVADs) have become more widely accepted. Although survival has improved with newer generation LVADs, multiple complications including pump failure due to outflow graft obstruction have become more clinically relevant. Patients with this complication will present with heart failure symptoms due to inadequate unloading of the left ventricle by LVAD. This rare complication often requires open surgical repair in an already high-risk patient population. Alternative means to relieve the obstruction via percutaneous intervention has been described in rare cases. We present two unique cases of LVAD outflow graft obstruction which were percutaneously treated successfully. This article illustrates a potential treatment strategy for outflow graft obstruction with stenting as an alternative to open-heart surgery.


Subject(s)
Heart Failure , Heart-Assist Devices , Ventricular Outflow Obstruction , Heart Failure/etiology , Heart Failure/therapy , Heart Ventricles/diagnostic imaging , Heart Ventricles/surgery , Heart-Assist Devices/adverse effects , Humans , Treatment Outcome , Ventricular Outflow Obstruction/diagnostic imaging , Ventricular Outflow Obstruction/etiology , Ventricular Outflow Obstruction/therapy
10.
Catheter Cardiovasc Interv ; 96(7): 1445-1453, 2020 12.
Article in English | MEDLINE | ID: mdl-33022100

ABSTRACT

OBJECTIVES: To assess the impact of right ventricular outflow tract (RVOT) stenting as the primary palliation in infants with complete atrioventricular septal defect with associated tetralogy of Fallot (cAVSD/TOF). BACKGROUND: Historically, palliation of symptomatic patients with cAVSD/TOF has been achieved through surgical systemic to pulmonary artery shunting. More recently RVOT stenting has evolved as an acceptable alternative in patients with tetralogy of Fallot. METHODS: Retrospective review of all patients with cAVSD/TOF who underwent RVOT stenting as palliation over a 13-year period from two large tertiary referral centers. RESULTS: Twenty-six patients underwent RVOT stenting at a median age of 57 days (interquartile range [IQR] 25.5-106.5). Median weight for stent deployment was 3.7 kg (IQR 2.91-5.5 kg). RVOT stenting improved oxygen saturations from a median of 72% (IQR 70-76%) to 90% (IQR 84-92%), p < .001. There was a significant increase in the median Z-score for both branch pulmonary arteries at median follow-up of 255 days (IQR 60-455). Eight patients required RVOT stent balloon dilatations and 8 patients required re-stenting for progressive desaturation. The median duration between reinterventions was 122 days (IQR 53-294 days). Four patients died during the follow-up period. No deaths resulted from the initial intervention. To date, definitive surgical intervention was achieved in 19 patients (biventricular repair n = 15) at a median age of 369 days (IQR 223-546 days). CONCLUSION: RVOT stenting in cAVSD/TOF is a safe and effective palliative procedure in symptomatic infants, promoting pulmonary artery growth and improving oxygen saturations.


Subject(s)
Cardiac Catheterization/instrumentation , Heart Septal Defects/therapy , Palliative Care , Stents , Tetralogy of Fallot/therapy , Ventricular Outflow Obstruction/therapy , Cardiac Catheterization/adverse effects , Cardiac Catheterization/mortality , England , Female , Heart Septal Defects/diagnostic imaging , Heart Septal Defects/mortality , Heart Septal Defects/physiopathology , Humans , Infant , Ireland , Male , Pulmonary Artery/growth & development , Recovery of Function , Retreatment , Retrospective Studies , Tetralogy of Fallot/diagnostic imaging , Tetralogy of Fallot/mortality , Tetralogy of Fallot/physiopathology , Time Factors , Treatment Outcome , Ventricular Function, Right , Ventricular Outflow Obstruction/diagnostic imaging , Ventricular Outflow Obstruction/mortality , Ventricular Outflow Obstruction/physiopathology
11.
Can J Cardiol ; 36(12): 1977.e5-1977.e8, 2020 12.
Article in English | MEDLINE | ID: mdl-32687863

ABSTRACT

Hemolytic anemia after mitral valve repair and ring annuloplasty is uncommon when compared with mitral valve replacement. In this report we present the case of a 67-year-old woman who had undergone mitral valve repair with a Duran band and developed hemolytic anemia. Most patients with severe hemolysis after mitral valve repair undergo reoperation. However, in the present case, the hemolysis was found to be secondary to systolic anterior motion of the mitral valve and left ventricular outflow tract obstruction and was treated without the need for reoperation.


Subject(s)
Anemia, Hemolytic , Metoprolol/administration & dosage , Mitral Valve Annuloplasty/adverse effects , Mitral Valve , Postoperative Complications , Ventricular Outflow Obstruction , Adrenergic beta-1 Receptor Antagonists/administration & dosage , Aged , Anemia, Hemolytic/diagnosis , Anemia, Hemolytic/etiology , Anemia, Hemolytic/therapy , Echocardiography, Transesophageal/methods , Female , Humans , Mitral Valve/diagnostic imaging , Mitral Valve/pathology , Mitral Valve/physiopathology , Mitral Valve Annuloplasty/methods , Mitral Valve Insufficiency/diagnosis , Mitral Valve Insufficiency/surgery , Mitral Valve Prolapse/diagnosis , Mitral Valve Prolapse/surgery , Postoperative Complications/blood , Postoperative Complications/etiology , Postoperative Complications/physiopathology , Postoperative Complications/therapy , Treatment Outcome , Ventricular Outflow Obstruction/diagnosis , Ventricular Outflow Obstruction/etiology , Ventricular Outflow Obstruction/physiopathology , Ventricular Outflow Obstruction/therapy
15.
Arch Cardiovasc Dis ; 113(2): 113-120, 2020 Feb.
Article in English | MEDLINE | ID: mdl-32081640

ABSTRACT

BACKGROUND: The indications for percutaneous pulmonary valve implantation (PPVI) have been extended to include large dysfunctional right ventricular outflow tracts (RVOTs). Prestenting of the RVOT is commonly performed before PPVI in order to ensure a stable landing zone. The AndraStent XXL (AndraMed GmbH, Reutlingen, Germany), a cobalt-chromium stent with semi-open cell design, has unique mechanical properties in this indication but is no longer available in France. AIMS: To assess the efficiency of AndraStent XXL before PPVI. METHODS: In this retrospective multicentre cohort study, 86 AndraStents XXL were implanted in 77 patients in 6 centres. RESULTS: PPVI was indicated mainly for pulmonary regurgitation (75.3%) in native or patched RVOT (88.3%). The stents were manually mounted on balloon catheters and delivered through sheaths using a conventional femoral approach. PPVI was performed successfully in 97.4% of patients after successful prestenting, generally during the same procedure (77.9%). There were no deaths associated with stent implantation, and four patients experienced five complications, mainly stent embolization, including one requiring surgery. Neither stent fracture nor dysfunction were observed in any patient during a mean follow-up of 19.2±8.7months. Stent analysis showed an excellent maximal stent expansion (97.1%) regardless of balloon size. A 22.3%±3.4 stent shortening with a 30mm balloon was observed. CONCLUSIONS: Implantation of large cobalt-chromium AndraStent XXL stents is efficient for prestenting before PPVI.


Subject(s)
Cardiac Catheterization/instrumentation , Heart Valve Prosthesis Implantation/instrumentation , Heart Valve Prosthesis , Pulmonary Valve Insufficiency/surgery , Pulmonary Valve/surgery , Stents , Ventricular Outflow Obstruction/therapy , Adolescent , Adult , Cardiac Catheterization/adverse effects , Child , Female , France , Heart Valve Prosthesis Implantation/adverse effects , Humans , Male , Middle Aged , Prosthesis Design , Pulmonary Valve/diagnostic imaging , Pulmonary Valve/physiopathology , Pulmonary Valve Insufficiency/diagnostic imaging , Pulmonary Valve Insufficiency/physiopathology , Recovery of Function , Retrospective Studies , Treatment Outcome , Ventricular Outflow Obstruction/diagnostic imaging , Ventricular Outflow Obstruction/physiopathology , Young Adult
16.
Int J Cardiol ; 304: 86-92, 2020 04 01.
Article in English | MEDLINE | ID: mdl-31983465

ABSTRACT

Patients with hypertrophic cardiomyopathy (HCM) exhibit a variable phenotype with ventricular hypertrophy as the cardinal manifestation and left ventricular (LV) outflow tract obstruction (LVOTO) as a key pathophysiologic determinant. Patients with severe LVOTO usually present with exertional dyspnea, exertional syncope, and heart failure symptoms, while successful relief of LVOTO by pharmacological or invasive interventions leads to a dramatic improvement in clinical status. Proper management of obstructive HCM remains challenging and poses numerous clinical dilemmas. Since the development of surgical myectomy over half a century ago, progress in the management of LVOTO in HCM has paralleled technological advances in genetic testing, cardiac imaging, arrhythmic prophylaxis, cardiac surgery and interventional cardiology. These changes have been incorporated in dedicated scientific guidelines on both sides of the Atlantic. However, either the 2011 American guidelines or the 2014 European guidelines remain largely based on expert consensus for lack of recommendations with level of evidence A regarding any of the treatment options commonly employed in HCM. Consequently, management of obstructive HCM patients remains largely subjective and dependent on clinical judgment, local expertise, and patient preference. Following the trend that has emerged for other cardiac diseases amenable to invasive interventions, adequate evaluation and management of obstruction in HCM today requires a multidisciplinary team capable of optimizing referral, choosing the best available options, minimizing complications and ensuring state-of-the-art results. The concept of an HCM Heart Team is coming of age. This review aims to provide an update of available pharmacologic and invasive options for the management of LVOTO in HCM, either in adulthood or in childhood, highlighting areas for multidisciplinary integration and future development.


Subject(s)
Cardiac Surgical Procedures , Cardiomyopathy, Hypertrophic , Heart Defects, Congenital , Heart Failure , Ventricular Outflow Obstruction , Adult , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/epidemiology , Cardiomyopathy, Hypertrophic/therapy , Humans , Ventricular Outflow Obstruction/diagnostic imaging , Ventricular Outflow Obstruction/therapy
18.
Int Heart J ; 60(6): 1435-1440, 2019 Nov 30.
Article in English | MEDLINE | ID: mdl-31735771

ABSTRACT

Hypertrophic cardiomyopathy with left ventricular (LV) mid-cavity obstruction and LV apical aneurysm is associated with high morbidity and mortality rates. However, consensus is lacking on the treatment modality for LV mid-cavity obstruction and LV apical aneurysm. Here, we report a case of reduced LV mid-cavity pressure gradient and symptoms, treated using permanent pacing. The effect of permanent pacing on pressure gradient and symptoms lasted for 4 years. As pacing is relatively non-invasive compared to surgical therapy, permanent pacing is a good option, especially in the elderly patients with LV mid-cavity obstruction and apical aneurysm.


Subject(s)
Cardiac Pacing, Artificial , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/therapy , Heart Aneurysm/complications , Ventricular Dysfunction, Left/complications , Ventricular Outflow Obstruction/complications , Aged , Cardiomyopathy, Hypertrophic/diagnosis , Female , Heart Aneurysm/diagnosis , Heart Aneurysm/therapy , Humans , Pacemaker, Artificial , Ventricular Dysfunction, Left/diagnosis , Ventricular Dysfunction, Left/therapy , Ventricular Outflow Obstruction/diagnosis , Ventricular Outflow Obstruction/therapy
19.
BMJ Case Rep ; 12(11)2019 Nov 28.
Article in English | MEDLINE | ID: mdl-31780619

ABSTRACT

Isolated dextrocardia is a congenital anomaly characterised by the normal position of the thoracic and abdominal viscera with a right cardiac apex. Left ventricular outflow tract obstruction (LVOTO) is a common structural manifestation of hypertrophic cardiomyopathy (HCM). A 65-year-old woman had worsening chest discomfort and dyspnoea on exertion. Chest CT angiography identified the isolated dextrocardia and HCM. Colour Doppler echocardiography showed mosaic flow in the LV outflow, indicating LVOTO. We performed alcohol septal ablation (ASA) under intracardiac echocardiography (ICE)-guided selective myocardial contrasting. This procedure improved provoked intra-LV pressure gradient by Valsalva manoeuvre and nitroglycerin injection from 136 to 50 mm Hg and her symptoms. The unique combination of isolated dextrocardia and left ventricular hypertrophy could have been involved in the formation of latent LVOTO. Even with the anomaly, contrast ICE made it possible to clarify the target septal left ventricular wall of ASA, and we could perform ASA safely.


Subject(s)
Ethanol/therapeutic use , Ventricular Outflow Obstruction/therapy , Aged , Dextrocardia/complications , Female , Heart Septum , Humans , Ventricular Outflow Obstruction/complications
20.
J Med Case Rep ; 13(1): 291, 2019 Sep 15.
Article in English | MEDLINE | ID: mdl-31521201

ABSTRACT

BACKGROUND: Subpulmonic membrane as a cause of right ventricular outflow tract obstruction in patients with concordant ventriculoarterial connection and intact ventricular septum is considered to be rare. Association with cor triatriatum dexter and success of subpulmonic balloon valvuloplasty have never been reported, at least to the best of our knowledge. CASE PRESENTATION: A 3-year-old Moroccan boy was referred to our tertiary care hospital with complaints of dyspnea on moderate exertion. A physical examination revealed parasternal lift, systolic thrill, and a 4/6 ejection systolic murmur, best heard over the left second intercostal space. His oxygen saturation was 99% on room air. Two-dimensional echocardiography showed a discrete circumferential membrane just below the pulmonic valve and a right atrial membrane. Continuous wave Doppler interrogation showed peak systolic pressure gradient of 85 mmHg across the subpulmonic membrane and no significant gradient across the right atrial membrane. Balloon dilation of the subpulmonic membrane was performed and the pressure gradient came down to 50 mmHg. During follow-up, he reported marked improvement in terms of exercise tolerance. Transthoracic echocardiography showed residual pressure gradient of approximately 40 mmHg across the membrane. Surgery resection of the two membranes was programmed, but he died after an extracardiac disease (appendicular peritonitis). CONCLUSIONS: Subpulmonic membrane as an isolated cause of right ventricular outflow tract obstruction is rare. Its association with cor triatriatum dexter is even less common. The result of percutaneous balloon valvuloplasty of subpulmonic membrane is an interesting alternative while waiting for surgery. Surgery is currently the preferred modality of treatment with the resection of both right atrial and subpulmonic membranes.


Subject(s)
Balloon Valvuloplasty , Cor Triatriatum , Pulmonary Valve Stenosis/therapy , Ventricular Outflow Obstruction/therapy , Child, Preschool , Echocardiography , Humans , Male , Pulmonary Valve Stenosis/diagnostic imaging , Radiography , Ventricular Outflow Obstruction/etiology
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