Congenital unicuspid aortic valve stenosis in siblings

We reported the case of unicuspid aortic valve in sibling, suggesting the familial incidence and genetic relation. A 41-year-old man (elder brother) with 79 mmHg of aortic valvular peak pressure gradient (peak PG) underwent aortic valve replacement with mechanical prosthetic valve (ATS 21 mm). The intraoperative finding showed the unicuspid aortic valve with one rudimentary commissure. A 37-year-old woman (sister) had been followed by echocardiography as bicuspid aortic valve since 10 years old. After 27 years, the peak PG had increased to 176 mmHg on preoperative echocardiography. The aortic valve replacement was performed. The bioprosthetic valve (MOSAIC 21 mm) was implanted supra-annularly because the patient required pregnancy. The intraoperative finding showed the unicuspid aortic valve with two rudimentary commissures and one opening of LCC–RCC commissure. The histopathological study of both patients confirmed as congenitally unicuspid aortic valve. In our best knowledge, this is a first report of unicuspid aortic valve in sibling.     

Early and mid-term results of isolated aortic valve neocuspidization in patients with aortic stenosis

Objectives

This study aimed to elucidate the surgical outcome of aortic valve neocuspidization (AVNeo) in patients with aortic stenosis (AS).

Methods

From December 2010 to June 2017, we performed AVNeo for aortic valve pathologies in 144 patients. Of them, we evaluated 57 patients with AS who underwent AVNeo. Their mean age was 77.5?±?8.8 years. Fifty-five patients had AS from degenerative changes, 1 from pericardium endocarditis, and 1 from prosthetic valve endocarditis. Forty patients had a tricuspid aortic valve, 1 had a unicuspid valve, 14 had a bicuspid valve, 1 had a quadricuspid valve, and 1 had postoperative aortic valve replacement (AVR). Preoperative echocardiography revealed an average peak pressure gradient of 89?±?32.9 mmHg and a mean pressure gradient of 52?±?18.8 mmHg. The surgical procedure complies with the 3 cuspid suturing to the aortic annulus with the glutaraldehyde-treated pericardium.

Results

There were no conversions to AVR. There were 2 noncardiac-related deaths owing to liver failure and sepsis. Postoperative echocardiography showed an average peak pressure gradient of 22?±?10.7 mmHg 1 week after the procedure and 19.2?±?9.7 mmHg 20 months after the procedure. Two patients underwent reoperation owing to infective endocarditis and recurrent aortic regurgitation. The mean follow-up period was 30.4?±?20.8 months. The freedom from reoperation rates was 98.1 and 95.3% at 12 and 81 months of follow-up, respectively.

Conclusions

AVNeo is suitable for patients with AS considering its early and mid-term outcomes. Verification of long-term outcomes and reliability is necessary.

     

Midterm outcomes after aortic valve neocuspidization with glutaraldehyde-treated autologous pericardium

Objective

We had previously reported the short-term results of the aortic valve neocuspidization (AVNeo) procedure. We have now evaluated the midterm results with the longest follow-up of 118 months.

Beta-blocker stress echocardiography in an aortic stenosis patient with associated left ventricular outflow tract obstruction

An 80-year-old man visited our hospital because of dyspnea on exertion from 6 months ago. Echo Doppler study showed severe calcification in the aortic valve with restricted movement and the sigmoid septum causing obstruction at the LV outflow tract (LVOT). Considering the aortic valve area (AVA) might have been inaccurately estimated, we carried out beta-blocker stress echocardiography. The transaortic pressure gradient and AVA were respectively calculated as 52 mmHg and 0.90 cm² before propranolol administration and as 64 mmHg and 0.86 cm² after propranolol administration. Thus, beta-blocker stress echocardiography may provide an accurate assessment of AS if the LVOT obstruction is concomitant.     

Aortic shear stress in patients with bicuspid aortic valve with stenosis and insufficiency

Objectives

Bicuspid aortic valve, characterized by valve malformation and risk for aortopathy, displays profound alteration in systolic aortic outflow and wall shear stress distribution. The present study performed 4-dimensional flow magnetic resonance imaging in patients with bicuspid aortic valve with right-left cusp fusion, focusing on the impact of valve function on hemodynamic status within the ascending aorta.

Reimplantation of the aortic valve at 20 years

Objective

To provide additional information on clinical and echocardiographic outcomes after reimplantation of the aortic valve (RAV) in patients with aortic root aneurysm.

Selection of prosthetic aortic valve and root replacement in patients younger than age 30 years

Objective

Long-term outcomes of prosthetic aortic valve/root replacement in patients aged 30 years or younger are not well understood. We report our single institutional experience in this young cohort.

Intracardiac echocardiography to diagnose pannus formation after aortic valve replacement

A 66-year-old female, under regular follow-up for 20 years after aortic valve replacement (19-mm Carbomedics), presented dyspnea on effort and hypotension during hemodialysis. A transthoracic echocardiogram showed elevation of transvalvular velocity up to 4 m/s, but the structure around the aortic prosthesis was difficult to observe due to artifacts. Fluoroscopy revealed normal motion of the leaflets of the mechanical valve. Intracardiac echocardiography (ICE) revealed a pannus-like structure in the left ventricular outflow tract. Transesophageal echocardiogram also revealed this structure. ICE can visualize structural abnormalities around a prosthetic valve after cardiac surgery even in patients in whom conventional imaging modalities failed.     

A missing leaflet: a rare cause of structural deterioration of a bovine pericardial aortic bioprosthesis—report of a case

We experienced a rare cause of aortic bioprosthesis deterioration in which one of the leaflets disappeared 8.7 years after primary aortic valve replacement (AVR) in a male octogenarian. Successful redo AVR with a 23-mm Magna EASE (Carpentier-Edwards, Irvine, CA) was performed. No embolic complications occurred. We were unable to identify the cause of this devastating complication. When heart failure symptoms develop in patients with bioprostheses, clinicians should therefore consider a diagnosis of acute progression of structural valve deterioration and follow the patient carefully with echocardiography.     

Early failure of quadricuspid aortic valve tricuspidization due to dehiscence of approximating coronary cusps

A 48-year-old woman diagnosed with aortic regurgitation showing a quadricuspid aortic valve (QAV) underwent repair with tricuspidization. Two non-coronary cusps (NCC) with fenestrations were detected intraoperatively. Tricuspidization of QAV was performed by approximating the two NCCs via interrupted sutures. The aortic annulus was plicated by subcommissural annuloplasty. The residual aortic regurgitation was mild, with a vena contracta of 2.1 mm. On postoperative day 5, echocardiography demonstrated a new regurgitation from the middle of the neo-NCC, and increased central regurgitation. Intraoperative findings showed the dehiscence of approximating sutures of the neo-NCC. Mechanical valve replacement was performed uneventfully.     

Early outcomes and hemodynamics after implantation of the Trifecta aortic bioprosthesis

Objective

The Trifecta valve is a recent, newly designed high performance valve, with few studies on the clinical and hemodynamic data. The purpose of this study was to evaluate the early clinical and echocardiographic results of the Trifecta valve.

Methods

Between April 2012 and December 2012, 23 consecutive patients underwent aortic valve replacement with the Trifecta valve in our institution. Clinical and hemodynamic data were prospectively recorded and hemodynamic performance was assessed by transthoracic echocardiography.

Results

Nine patients were male and the overall mean age was 75 ± 9 years. Twenty patients suffered aortic stenosis, and 3 suffered aortic insufficiency. Prosthesis sizes implanted were: 19 mm (n = 4), 21 mm (n = 12), and 23 mm (n = 7). There were no 30-day deaths and no valve-related events during follow-up, except for 1 postoperative stroke. The mean postoperative transprosthetic pressure gradient was 10.0 ± 1.4, 9.6 ± 3.6, and 7.1 ± 3.6 mmHg, and the effective orifice area was 1.45 ± 0.13, 1.68 ± 0.16, and 1.90 ± 0.28 cm², for valve sizes 19, 21, and 23 mm, respectively. One patient had moderate prosthesis–patient mismatch. No moderate to severe aortic regurgitation was observed. The mean pressure gradient in aortic stenosis patients decreased significantly from 49.9 ± 20.7 to 8.9 ± 3.6 mmHg (p < 0.001). Left ventricular mass index in all patients decreased significantly from 142.0 ± 33.6 to 115.4 ± 26.4 g/m² (p < 0.001).

Conclusions

The Trifecta aortic bioprosthesis provided satisfactory early outcomes and hemodynamic function.     

A case of concomitant repair of aortic regurgitation due to congenital aortic quadricuspid valve and coronary artery occlusive disease

A case of rare congenital anomaly of quadricuspid aortic valve and coexisting coronary artery occlusive disease of a 60-year-old female was presented. She was admitted to our hospital for heart murmur and angina pectoris. The echocardiography and aortography showed quadricuspid aortic valve and aortic valve regurgitation. The coronary arteriography revealed the presence of triple vessels disease. Successful aortic valve replacement and Coronary bypass grafting were performed simultaneously.     

The early and mid-term results of mitral valve repair for mitral regurgitation in children

Purpose

To review the surgical techniques and mid-term results of mitral valve repair in children with moderate or severe mitral regurgitation (MR).

Methods

One hundred and seven children with moderate or severe MR, aged 19.6 ± 8.5 months, were enrolled in this study. The surgical techniques used for mitral valve repair varied according to the mitral valve morphology, and included annuloplasty, annuloplasty ring, cleft closure, reconstruction of the posterior leaflet, etc. The concomitant cardiac anomalies were treated simultaneously. The results of repair were evaluated by transesophageal echocardiography performed during the operation and by serial transthoracic echocardiography performed during the follow-up.

Results

One hundred and six cases had no more than mild regurgitation intraoperatively, whereas only one case had moderate regurgitation. This patient underwent redo repair immediately, and the subsequent regurgitation was trivial. The in-hospital mortality rate was 0.9 % (1/107). The average follow-up was 46.5 ± 8.2 months. One patient died of heart failure 10 months postoperatively. The freedom from moderate or severe regurgitation after mitral valve repair was 92.3 ± 3.3 %.

Conclusion

Pediatric patients with moderate or severe MR require early surgical treatment. The early and mid-term results of mitral valve repair in pediatric patients were satisfactory.     

Active infective endocarditis due to Erysipelothrix rhusiopathiae: zoonosis caused by vancomycin-resistant gram-positive rod

A 42-year-old female who was a voluntary worker in a school for handicapped children was referred to us for surgery for active infective endocarditis. Trans-esophageal echocardiography showed 2 large mobile vegetations on the aortic valve and severe aortic regurgitation. Aortic valve replacement was performed to prevent septic embolism and deterioration of congestive heart failure. The empiric therapy with vancomycin, ampicillin, and gentamycin was initiated because a pathogen was not identified. But Erysipelothrix rhusiopathiae (gram-positive rod) was isolated on the 4th day after surgery. The target therapy with penicillin G and clindamycin was started and continued for 4 weeks after surgery. The inflammatory parameters improved steadily and the patient was discharged on the 36th day after surgery. Infective endocarditis due to gram-positive rods can be easily mistaken for streptococci or dismissed as a skin contamination. But, E. rhusiopathiae endocarditis should be considered in the differential diagnosis.     

Impact of patient-specific morphologies on sinus flow stasis in transcatheter aortic valve replacement: An in vitro study

Objective

The goal of this study is to evaluate how sinus flow patterns after transcatheter aortic valve replacement in realistic representative patient roots vary. Sinus flow can affect transcatheter aortic valve operation and likely leaflet thrombosis occurrence due to stasis and poor washout. How the interaction between transcatheter aortic valve and representative patient aortic roots affects sinus hemodynamics is important to establish for future individualization of transcatheter aortic valve replacement therapy.

The impact of age on the postoperative response of the diastolic function and left ventricular mass regression after surgical or transcatheter aortic valve replacement for severe aortic stenosis

Purpose

We examined the impact of advanced age on left ventricular mass regression and the change in the diastolic function after aortic valve replacement in patients with aortic stenosis.

Methods

The present study included 129 patients who underwent either surgical or transcatheter aortic valve replacement and 1-year postoperative echocardiography. The patient characteristics and echocardiographic findings were compared between patients who were <80 years of age (group Y: n = 69) and those who were ≥80 years of age (group O: n = 60).

Results

Preoperative echocardiography revealed that although the left ventricular mass was similar between the groups, the patients in group O had more severe diastolic dysfunction in comparison to those in group Y. Postoperatively, left ventricular mass regression was significantly greater (p = 0.02) and diastolic dysfunction was less prevalent in group Y (p = 0.02) in comparison to group O. The change in E/e′ was significantly correlated with the left ventricular mass regression in group Y (p = 0.02), but not in Group O (p = 0.21).

Conclusions

The patients in group O were less susceptible to improvements in myocardial remodeling and the diastolic function in comparison to those in group Y. The altered physiological response to aortic valve replacement might help to determine the appropriate timing of surgery in elderly patients.

     

Is routine computed tomography angiography justified in patients undergoing aortic valve replacement for bicuspid aortic valve disease?

Background

Role of Computed Tomography Angiography (CTA) in patients with Bicuspid Aortic Valve (BAV) undergoing Aortic Valve Replacement (AVR) needs assessment.

Patients and Methods

After echocardiography, 54 patients with BAV were referred for AVR. CTA was performed routinely. Pre-operative characteristics, echocardiographic and CTA findings, and details of surgery were obtained.

Results

The study population had 54 subjects (48 males). Median age was 35.5 years (range 7 to 78 years), and median weight was 57.5 Kg (range 14 to 83 kg). On echocardiography, aortic sinus diameter ranged from 13 to 38 mm (median 28 mm). In none of the patients, ascending aorta was reported to be dilated. On CT angiography, the sinus diameter ranged from 16 to 46 mm (median 35 mm). Sinus diameter was ≥40 mm in 13 patients. The sinus diameter on echocardiography was within the range of 0 to 2 mm of CT angiographic estimates in 31 patients, within 2.1 to 5 mm in 22 patients, and more than 5 mm in one patient. The ascending aortic diameter ranged from 19 to 70 mm (median 43 mm). In 26 patients, ascending aortic diameter was ≥45 mm. In 12 patients, the proximal arch diameter was ≥40 mm. In two patients, the distal ascending aorta and proximal arch were aneurysmally dilated (48 mm and 57 mm). In 12 patients, the ascending aorta was dilated (≥ 45 mm) without any sinus dilatation. In one patient, the distal ascending aorta and proximal arch were aneurysmally dilated (57 mm) without any proximal dilatation. Based on CT angiographic findings, 25 patients (46.3 %) underwent additional aortic replacement in the form of Bentall’s procedure (n?=?7), Bentall’s + Hemiarch replacement (n?=?6), aortoplasty (n?=?5), Wheat procedure (n?=?6) and Wheat procedure?+?Hemiarch replacement (n?=?1).

Conclusion

CT angiography is justified as a routine pre-operative evaluation tool in all patients with BAV who are undergoing open heart surgery for significant aortic valve dysfunction.     

Repeat surgical intervention after aortic repair for acute Stanford type A dissection

Objective

This study aimed to evaluate the outcomes of repeat interventions on the aorta and aortic valve after surgery for acute Stanford type A aortic dissection.

Methods

The hospital records of patients who underwent repeat surgical intervention between April 2011 and March 2017 for late complications after acute type A aortic dissection repair were retrospectively reviewed.

Results

We identified 17 patients with mean age of 62?±?8 years; 13 were men. The mean interval from the initial emergency aortic repair to the repeat intervention was 5.8?±?5.4 years (range 133 days–16.6 years). Ten patients had dilatation or rupture of the residual type B aortic dissection; six of them had retrograde type A aortic dissection at the onset and did not undergo resection of the primary entry. Five patients had a pseudoaneurysm at the anastomosis; four of them were receiving anticoagulation medication. Three patients had aortic regurgitation; two of them were associated with the gelatin-resorcinol-formaldehyde glue that was used during the initial surgery. There was no early mortality after repeat intervention and no late death after a mean follow-up period of 3.3?±?2.0 years.

Conclusions

Repeat surgical intervention on the aorta and aortic valve after repair of acute type A aortic dissection had favorable early and mid-term outcomes and was not associated with early or late death. Long-term follow-up with imaging and echocardiography was considered to be essential for early detection of residual type B dilatation, anastomotic pseudoaneurysm, and aortic regurgitation after initial aortic repair.

     

Aortic valve reconstruction in Rubinstein-Taybi-syndrome: the valuable aid of transesophageal echocardiography

A fourteen year old boy with Rubinstein-Taybi-syndrome presented with a severe congenital subvalvular fibrous aortic stenosis with associated aortic regurgitation. Transthoracic echocardiographic imaging and left heart angiography showed a transvalvular peak systolic pressure gradient of 90 mmHg and a regurgitant fraction of 30%. The surgical treatment consisted of resection of the subvalvular fibrous tissue and subsequent aortic valvuloplasty. Intraoperative transesophageal echocardiography revealed a wide systolic opening of the aortic valve and good coaptation of the aortic valve leaflets in diastole. Two-dimensional color-coded and contrast echocardiography were successfully used to confirm a satisfactory reconstruction. Transesophageal echocardiography therefore represents a valuable tool in the assessment of the morphologic and haemodynamic status, especially in rare cases of congenital heart disease in older children.     

Survival and reoperation pattern after 20 years of experience with aortic valve–sparing root replacement in patients with tricuspid and bicuspid valves

Objective

Remodeling or reimplantation are established operative techniques of aortic valve–sparing root replacement. Long-term follow-up is necessary comparing tricuspid and bicuspid aortic valves.