Aorto-left ventricular tunnel: case series of a rare disease

2020 ◽  
pp. 1-5
Author(s):  
Obayda M. Diraneyya ◽  
Fahad Alhabshan ◽  
Abdullah Alghamdi ◽  
Hussein Moafa ◽  
Mohammed Alnasef ◽  
...  

Abstract Introduction: Aorto-left ventricular tunnel is a rare disease that can cause significant morbidity early in life due to volume overload and left ventricular failure. Surgical intervention is usually curative with minimal early complications. However, long-term effects and outcome are not fully determined. Objective: We are reporting a case series of this rare CHD with its long-term outcome. Methods: We conducted a retrospective analysis of all children from birth to 14 years of age who were admitted between 2001 and 2020 with the diagnosis of aorto-left ventricular tunnel. Demographic, echocardiographic, and perioperative data were collected and reviewed. The pre-operative data were compared with data reviewed on the last outpatient follow-up. Results: Total of five patients fulfilled our inclusion criteria. Three patients were diagnosed after auscultating an incidental murmur, one had symptoms of congestive heart failure, and one had an abnormal fetal echocardiogram. Echocardiography demonstrated stenotic and regurgitant aortic valve with severely depressed left ventricle function in two patients, one of them with also single left coronary artery. The other three patients had normal aortic valve structure and normal ventricular function. All five patients had surgical repair, two by patch closure at aortic end of aorto-left ventricular tunnel, two by patch closure at both aortic and left ventricular ends, and one by aortic root replacement using a homograft. During follow-up, there was no residual aorto-left ventricular tunnel in any of our five cases, two had moderate aortic regurgitation and one had moderate residual aortic stenosis. Conclusions: Spectrum of presentation for aorto-left ventricular tunnel varies from an occult lesion to frank left heart failure due to volume or less commonly, pressure overload. Early surgical repair is recommended and is usually associated with complete resolution. Long-term follow-up is recommended for aortic root dilatation and aortic valve competency, as valve function need to be addressed in a timely manner to avoid further complications.

Author(s):  
Jing Sun ◽  
Hongxia Qi ◽  
Hongyuan Lin ◽  
Wenying Kang ◽  
Shoujun Li ◽  
...  

Abstract OBJECTIVES Aortico-left ventricular tunnel (ALVT) is an extremely rare, abnormal paravalvular communication between the aorta and the left ventricle. Few studies have identified the characteristics and long-term prognosis associated with ALVT. METHODS The data of 31 patients with ALVT from July 2002 to December 2019 were reviewed. Echocardiography was performed in all patients during the follow-up period. RESULTS The median age of the patients was 11.5 years. Bicuspid aortic valve and dilatation of the ascending aorta were found in 13 patients, respectively. The aortic orifice in 20 patients showed a close relation to the right sinus and the right–left commissure. Of the 31 patients, 26 were operated on. Mechanical valve replacement was performed in 4 patients and aortic valve repair, in 6 patients. Ascending aortoplasty was performed in 5 patients and aortic replacement was done in 2 patients. One patient died of ventricular fibrillation before the operation. Follow-up of the remaining 30 patients ranged from 1 to 210 months (median 64 months). There were 4 deaths during the follow-up period: 1 had mechanical valve replacement and 3 did not undergo surgical repair. In the 26 patients without aortic valve replacement, 6 had severe regurgitation and 2 had moderate regurgitation. In the 28 patients without replacement of the ascending aorta, 11 had continued dilatation of the ascending aorta, including those who had aortoplasty. CONCLUSIONS The aortic orifice of ALVT showed an association with the right sinus and the right–left commissure. For patients who did not have surgery, the long-term survival rate remained terrible. Surgical closure should be done as soon as possible after ALVT is diagnosed. The main long-term complications after surgical repair included aortic regurgitation and ascending aortic dilatation.


2020 ◽  
Vol 11 (5) ◽  
pp. 611-618
Author(s):  
Maria Rodriguez ◽  
Anahita Malvea ◽  
Dayre McNally ◽  
Vid Bijelic ◽  
Ming Guo ◽  
...  

Background: Pediatric aortic root dilatation is a life-threatening condition that lacks guidelines for surgical management. We aimed to analyze the data on aortic valve interventions during root surgery to guide decision-making. Methods: A search was performed of MEDLINE, Embase, CENTRAL, ClinicalTrials.gov , and WHO ICTRP. Citations were screened in duplicate and independently to identify randomized controlled trials, cohorts, and case series involving populations aged 0 to 18 years, who received valve-sparing and valve-replacing aortic root surgeries between 1999 and 2019. Outcomes considered included mortality (perioperative, one year, five year), reintervention rates. Results: After duplicate removal, 689 citations were screened through abstract and full text review, identifying five eligible studies. All five were observational studies evaluating valve-sparing procedures. There were 81 patients with a mean study age range of 9.9 to 13.9 years. Both reimplantation (74%) and remodeling (26%) subtypes were done. Range of mean duration of follow-up was 1.2 to 4.4 years. There was no mortality reported until the one-year follow-up period. The long-term mortality rate was calculated as 0.02 per patient-year (95% CI: 0.01-0.05). The long-term reintervention rate was 0.08 per patient-year (95% CI: 0.05-0.13). Conclusions: There is limited experience on aortic valve intervention during aortic root surgery in children. Single-arm studies on valve-sparing surgeries show excellent survival up to one year. Mortality and reintervention rates increase in the longer term. The small sample size and lack of controlled studies do not allow for direct comparisons between procedure types.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
S Zhang ◽  
X Xie ◽  
C He ◽  
X Lin ◽  
M Luo ◽  
...  

Abstract Background Late left ventricular remodeling (LLVR) after the index acute myocardial infarction (AMI) is a common complication, and is associated with poor outcome. However, the optimal definition of LLVR has been debated because of its different incidence and influence on prognosis. At present, there are limited data regarding the influence of different LLVR definitions on long-term outcomes in AMI patients undergoing percutaneous coronary intervention (PCI). Purpose To explore the impact of different definitions of LLVR on long-term mortality, re-hospitalization or an urgent visit for heart failure, and identify which definition was more suitable for predicting long-term outcomes in AMI patients undergoing PCI. Methods We prospectively observed 460 consenting first-time AMI patients undergoing PCI from January 2012 to December 2018. LLVR was defined as a ≥20% increase in left ventricular end-diastolic volume (LVEDV), or a >15% increase in left ventricular end-systolic volume (LVESV) from the initial presentation to the 3–12 months follow-up, or left ventricular ejection fraction (LVEF) <50% at follow up. These parameters of the cardiac structure and function were measuring through the thoracic echocardiography. The association of LLVR with long-term prognosis was investigated by Cox regression analysis. Results The incidence rate of LLVR was 38.1% (n=171). The occurrence of LLVR according to LVESV, LVEDV and LVEF definition were 26.6% (n=117), 31.9% (n=142) and 11.5% (n=51), respectively. During a median follow-up of 2 years, after adjusting other potential risk factors, multivariable Cox regression analysis revealed LLVR of LVESV definition [hazard ratio (HR): 2.50, 95% confidence interval (CI): 1.19–5.22, P=0.015], LLVR of LVEF definition (HR: 16.46, 95% CI: 6.96–38.92, P<0.001) and LLVR of Mix definition (HR: 5.86, 95% CI: 2.45–14.04, P<0.001) were risk factors for long-term mortality, re-hospitalization or an urgent visit for heart failure. But only LLVR of LVEF definition was a risk predictor for long-term mortality (HR: 6.84, 95% CI: 1.98–23.65, P=0.002). Conclusions LLVR defined by LVESV or LVEF may be more suitable for predicting long-term mortality, re-hospitalization or an urgent visit for heart failure in AMI patients undergoing PCI. However, only LLVR defined by LVEF could be used for predicting long-term mortality. FUNDunding Acknowledgement Type of funding sources: None. Association Between LLVR and outcomes Kaplan-Meier Estimates of the Mortality


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ethan J Rowin ◽  
Barry J Maron ◽  
Tammy S Haas ◽  
John R Lesser ◽  
Mark S Link ◽  
...  

Background: Increasing penetration of high spatial resolution cardiovascular magnetic resonance (CMR) imaging into routine cardiovascular practice has resulted in more frequent identification of a subset of hypertrophic cardiomyopathy (HCM) patients with thin-walled, scarred left ventricular (LV) apical aneurysms. Prior experience involved relatively small numbers of patients with short follow-up and therefore the risk associated with this subgroup remains incompletely defined. Therefore, we assembled a large HCM cohort with LV apical aneurysms and long-term follow-up in order to clarify clinical course and prognosis. Methods and Results: Of 2,400 HCM patients, 60 (2.5%) were identified by CMR with LV apical aneurysm, 24 to 86 years of age, including 19 (32%) <45 years old; 70% male, and followed for 5.6 ± 3.5 years. Over the follow-up period, 24 patients experienced 31 adverse disease-related complications including: appropriate implantable cardioverter-defibrillator discharge for VT/VF (n=11), received or listed for heart transplant (n=6), heart failure death (n=5), nonfatal thromboembolic events (n=4), resuscitated out-of-hospital cardiac arrest (n=3), and sudden death (n=2). In addition, an intracavitary thrombus was identified in the apical aneurysm in 9 patients without a thromboembolic history. Combined HCM-related death and aborted life threatening event rate was 8.6% per year, nearly 6-fold greater than the 1.5% annual mortality rate reported in the general HCM population. Conclusions: Patients with LV apical aneurysms represent a high-risk subgroup within the diverse HCM spectrum, associated with substantial increased risk for disease-related morbidity and mortality, including advanced heart failure, thromboembolic stroke and sudden death. Identification of this unique HCM phenotype should prompt consideration for primary prevention ICD, and anticoagulation for stroke prophylaxis.


Author(s):  
Guglielmo Stefanelli ◽  
Fabrizio Pirro ◽  
Vincenzo Smorto ◽  
Alessandro Bellisario ◽  
Emilio Chiurlia ◽  
...  

Objective Stentless aortic valves have shown superior hemodynamic performance and faster left ventricular mass regression compared to stented bioprostheses. Yet, controversies exist concerning the durability of stentless valves. This case-matched study compared short- and long-term clinical outcomes of stentless LivaNova-Sorin Pericarbon Freedom™ (SPF) and stented Carpentier-Edwards Perimount (CEP) aortic prostheses. Methods From 2003 through 2006, 134 consecutive patients received aortic valve replacement with SPF at our institution. This cohort was matched, according to 20 preoperative clinical parameters, with a control group of 390 patients who received CEP prosthesis during the same time. The resulting 55 + 55 matched patients were analyzed for perioperative results and long-term clinical outcomes. Results Early mortality was 0% for both groups. Lower transvalvular gradients were found in the SPF group (10.6 ± 2.9 versus 15.7 ± 3.1 mmHg, P < 0.001). Overall late mortality (mean follow-up: 10.03 years) was similar for both groups (50.1% versus 42.8%, P = 0.96). Freedom from structural valve degeneration (SVD) at 13 years was similar for both groups (SPF = 92.3%, CEP = 73.9%, P = 0.06). Freedom from aortic valve reinterventions did not differ (SPF = 92.3%, CEP = 93.5%, P = 0.55). Gradients at 13-year follow-up remained significantly lower in SPF group (10.0 ± 4.5 versus 16.2 ± 9.5 mmHg, P < 0.001). Incidence of acute bacterial endocarditis (ABE) and major adverse cardiovascular and cerebrovascular events (MACCE) was similar. Conclusions SPF and CEP demonstrated comparable long-term outcomes related to late mortality, SVD, aortic valve reinterventions, and incidence of ABE and MACCE. Superior hemodynamic performance of SPF over time can make this valve a suitable choice in patients with small aortic root and large body surface area.


2020 ◽  
Vol 21 (3) ◽  
pp. 807 ◽  
Author(s):  
Iwona Świątkiewicz ◽  
Przemysław Magielski ◽  
Jacek Kubica ◽  
Adena Zadourian ◽  
Anthony N. DeMaria ◽  
...  

Acute ST-segment elevation myocardial infarction (STEMI) activates inflammation that can contribute to left ventricular systolic dysfunction (LVSD) and heart failure (HF). The objective of this study was to examine whether high-sensitivity C-reactive protein (CRP) concentration is predictive of long-term post-infarct LVSD and HF. In 204 patients with a first STEMI, CRP was measured at hospital admission, 24 h (CRP24), discharge (CRPDC), and 1 month after discharge (CRP1M). LVSD at 6 months after discharge (LVSD6M) and hospitalization for HF in long-term multi-year follow-up were prospectively evaluated. LVSD6M occurred in 17.6% of patients. HF hospitalization within a median follow-up of 5.6 years occurred in 45.7% of patients with LVSD6M vs. 4.9% without LVSD6M (p < 0.0001). Compared to patients without LVSD6M, the patients with LVSD6M had higher CRP24 and CRPDC and persistent CRP1M ≥ 2 mg/L. CRP levels were also higher in patients in whom LVSD persisted at 6 months (51% of all patients who had LVSD at discharge upon index STEMI) vs. patients in whom LVSD resolved. In multivariable analysis, CRP24 ≥ 19.67 mg/L improved the prediction of LVSD6M with an increased odds ratio of 1.47 (p < 0.01). Patients with LVSD6M who developed HF had the highest CRP during index STEMI. Elevated CRP concentration during STEMI can serve as a synergistic marker for risk of long-term LVSD and HF.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Z P Jing ◽  
J X Feng ◽  
X H Bao ◽  
T Li ◽  
Y Zhao ◽  
...  

Abstract Aims The possibility of endovascular reconstruction of aortic valve, sinus of Valsalva, and ascending aorta by a minimal-invasive single endograft has not been proven in vivo. Combining our own long-term experiences from transcatheter aortic valve replacement (TAVR) and Thoracic Endovascular Repair (TEVAR) for ascending and arch dissection, we designed the special endo-graft: a novel one-piece valved-fenestrated-bifurcated endografting, and tried to endovascularly reconstruct the area from Left ventricular outflow tract to aortic arch in animal experiments. Methods and results For 20 healthy adult female pigs weighed between 62.3±2.2 kilograms, we did aortic compute tomography angiography (CTA) examinations and measured morphologic parameters of aortic root. Then we accordingly customized the valved-fenestrated-bifurcated endograft. The endograft was delivered through transapical access and endovascularly reconstructed the segment from aortic valve to proximal part of aortic arch. The overall technical success rate was 95% because of one case of delivery system failure. Instant transesophageal echography (TEE) and aortic CTA confirmed ideal position of the endograft, satisfactory function of aortic valve, and the patency of coronary arteries in all subjects. During follow-up, 12 subjects were sacrificed according to the plan and seven were followed up for 8.1±3.6 months. There was one unplanned death of cardiac infection (unplanned mortality: 5.3%). Follow-up re-examinations (aortic CTA, cardiac ultrasound, and electrocardiogram) found no adverse events. Among 12 sacrificed subjects, there was no evidence of fenestrations alignment lost and no myocardial ischemia according to the pathological analysis. Conclusion The novel one-piece valved-fenestrated-bifurcated endografting might be feasible for minimal-invasive reconstruction of aortic root in animal models, thus provided a prospect to simultaneously treat pathologies involving aortic valve and aortic root in endovascular way.


2008 ◽  
Vol 34 (3) ◽  
pp. 583-588 ◽  
Author(s):  
Feyzan Özaslan ◽  
Thomas Wittlinger ◽  
Nadejna Monsefi ◽  
Tamimount Bouhmidi ◽  
Sinthu Theres ◽  
...  

Author(s):  
Jun Ho Lee ◽  
Ji-Hyuk Yang ◽  
Pyo Won Park ◽  
Jinyoung Song ◽  
June Huh ◽  
...  

Background Several reports described the repair of sinus of Valsalva aneurysms (SVAs); however, there is still debate regarding the optimal method of operation. We investigated the determinants of the development of significant aortic regurgitation (AR) and long-term survival after surgical repair. Methods Between January 1995 and December 2016, 71 patients (31 females; median age: 33.3 years) underwent surgical SVA repair with (n = 60) or without (n = 11) rupture. Aortic valvuloplasty (AVP) was performed using Trusler's technique in 28 patients (39.4%), and 11 patients (15.5%) underwent aortic valve replacement during the first operation. Results There was no early mortality, and three deaths occurred during follow-up (median: 65.4 months). Patients with grade II preoperative AR who underwent AVP tended to develop significant postoperative AR, but freedom from significant AR did not differ statistically (p = 0.387). Among patients who underwent AVP, freedom from significant AR did not differ statistically between those with grades I and II and those with grades III and IV (p = 0.460). Conclusion Surgical repair of SVA with or without rupture can be performed safely using the dual approach technique. Concomitant aortic valve repair can be performed without difficulty and should be recommended not only for patients with moderate or severe preoperative AR (grades III and IV) but also for those with minimal or mild preoperative AR (grades I and II), whose aortic valve geometry needs correction.


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