Critical Aortic Stenosis Research Articles

Fetal Cardiac Intervention：過去・現在・未来

Fetal cardiac intervention has developed over the last two decades in Europe and North America to prevent irreversible deterioration in the fetal heart caused by morphological cardiac malformation. Technique, patient selection, and effects of the intervention have been clarified through challenges faced by fetal cardiologists worldwide. The intervention is performed by direct puncture of the fetal heart through the maternal abdomen. The procedure is significantly invasive for the fetus and requires a high level of surgical skill. Severe congenital heart diseases, including critical aortic stenosis, pulmonary atresia with intact ventricular septum, and hypoplastic left heart syndrome with highly restrictive foramen ovale, are targets for the intervention. Fetal cardiac intervention is an important treatment for severe congenital heart disease in utero and is desired early introduction into clinical practice in Japan.

Neonatal Aortic Stenosis Is a Surgical Disease.

Neonates with critical aortic stenosis represent a challenging group of patients with severe obstruction at a valvar level and with symptoms of heart failure. If biventricular repair is chosen, open valvotomy (OV) has been firmly established as the most effective initial treatment. In comparison with blind ballooning, OV, with exact splitting of fused commissures and shaving of obstructing nodules, can produce a better valve with a maximum valve orifice, without causing regurgitation. Thus, predictable and consistent early and longer-lasting results in any type of valve morphology are provided. Clearly superior results can be achieved in a tricuspid valve arrangement. OV not only offers a high survival benefit in the long run, but also a high quality of life, by minimizing re-interventions and preserving the native aortic valve in the majority of patients.

Metastatic pulmonary calcification: ‘crazy paving’ pattern

A 60-year-old man was admitted under the cardiology team for increasing shortness of breath over the past two months. His medical history of note included stable renal transplant 8 years...

Characterising adequacy or inadequacy of the borderline left ventricle: what tools can we use?

Borderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone's complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.

Hybrid Procedure for Neonates With Hypoplastic Left Heart Syndrome at High-Risk for Norwood: Midterm Outcomes

Hybrid procedure offers patients with severe congenital heart disease an alternative initial procedure to conventional surgical reconstruction. We report the midterm outcomes of a cohort of neonates who had a hybrid procedure for variants of hypoplastic left heart syndrome because they were at high risk for the Norwood procedure. Between December 2005 and January 2013, 41 neonates underwent bilateral pulmonary artery banding followed by ductal stenting by means of a sternotomy at a median age of 6 days (range, 2 to 18 days) and weight of 2.6 kg (range, 1.5 to 3.7 kg). Thirty-five patients had hypoplastic left heart syndrome, and 6 patients had critical aortic stenosis with hypoplastic left ventricle. Primary indications for the hybrid procedure were low birth weight in 17 patients, hypoplastic left ventricle with the possibility of later biventricular repair in 6 patients, intact or near-intact atrial septum in 5 patients, and poor patient condition in 13 patients. Echocardiographic, angiographic, operative, and clinical data were reviewed. Outcomes were summarized with descriptive statistics and risk factors for mortality identified. All but 6 patients had an antenatal diagnosis, and 24 patients were from other congenital cardiac centers. Nine patients had perioperative balloon aortic valvuloplasty, 1 patient had fetal balloon aortic valvuloplasty, and 17 patients had intervention to their atrial septum (41.4%). There were 9 inpatient deaths (21.9%) and 4 interstage deaths (9.8%) after the hybrid procedure. Twenty-eight patients subsequently underwent either the Norwood procedure (11 patients), combined stage I and II (14 patients), or biventricular repair (3 patients). No patient had heart transplantation. Among the patients who had combined stage I and II as a second procedure after the hybrid procedure, there were 2 early deaths, 1 late death before the Fontan, and 1 late death after the Fontan completion after combined stage I and II. All patients who had subsequent Norwood procedure were midterm survivors. Three of the 4 patients who had biventricular repair were midterm survivors. Overall survival was 56.1% at a median follow-up of 32.0 months. By univariate analysis, patient factors, intact or near-intact atrial septum, and aortic atresia were associated with nonsurvival. Hybrid procedure as an alternative to the Norwood procedure offers good midterm survival in patients deemed at high risk for neonatal reconstruction.

Endovascular treatment in patients with combined pathology

Aortic valve stenosis is one of the most common diseases from the group of acquired heart disease, especially in elderly patients. Aortic valve replacement has become the “gold standard” and gives excellent results in all age groups of patients. For aortic valve replacement is sent to only one third of patients due to high risk due to age, severity of the defect, reduced contractility of the left ventricle, pulmonary hypertension and other comorbidities. One of the attempts to reduce the frequency of complications and mortality in this group of patients, and subsequently to make it possible to perform other surgical interventions, including aortic valve replacement, which is considered inoperable patients is holding a less traumatic surgery - aortic balloon catheter valvuloplasty. In the clinical observation deals with the case of heart disease - critical aortic stenosis, ischemic heart disease complicated by pulmonary hypertension and severe heart failure, in combination with gynecological diseases (uterine fibroids, endometrial hyperplasia in postmenopausal women), complicated by uterine bleeding and hemorrhagic anemia. Consider the risks of different surgical interventions. The possibility of modern endovascular care of patients in this situation simultaneously perform operations on uterine artery embolization, coronary angioplasty with stenting and aortic catheter balloon valvuloplasty. Also shown is the nearest and remote results of operative intervention.

Prenatally diagnosed critical valvular aortic stenosis

Prenatally diagnosed critical valvular aortic stenosis

Gaucher disease causing sudden cardiac death

Abstract A 17-year-old male patient with Gaucher disease was presented to our institution complaining of rapid irregular palpitations. Echocardiography showed the presence of critical aortic stenosis due to Gaucher disease.

Is Aortic Valve Leaflet Morphology Predictive of Outcome in Pediatric Aortic Valve Stenosis?

Initial palliative treatment of congenital aortic valve stenosis consists of either surgical commissurotomy or transcatheter balloon valvuloplasty. The basis for selection of primary approach usually is ill defined. It was hypothesized that aortic valve characteristics may be helpful in directing choice of intervention. This study assesses the impact of valve morphology on time to reintervention after catheter- or surgical-based therapy. A retrospective review of preprocedural echocardiographic aortic outflow characteristics was compared with outcomes of primary surgical or balloon valve interventions. Characteristics studied included (1) annular dimension; (2) leaflet number; (3) leaflet excursion; and (4) degree of leaflet coaptation. Patients included those <20 years with a primary diagnosis of aortic stenosis (AS) and no other hemodynamically significant lesions at our institutions from 2000 to 2011. A total of 102 patients were included, 31 classified as having critical and 71 as having noncritical aortic stenosis. Of the patients, 79 were male, and 50 underwent primary catheter intervention. Echo parameters were compared with procedural outcome as defined by death or need for reintervention. Receiver operator curves were utilized to determine the point within each morphologic feature where the greatest difference occurred. This was utilized as the distinguishing point within each the morphologic group. Analysis was conducted separately for critical and noncritical aortic stenosis. Kaplan-Meier analysis demonstrated no significant difference in time to reintervention or death whether initial palliation consisted of surgical commissurotomy or balloon valvuloplasty with respect to any of the morphologic characteristics studied. Patients with AS do equally well with surgical commissurotomy or balloon valvuloplasty as initial palliation. This holds true for those with either critical or noncritical aortic stenosis. Valve morphology did not help in selection of initial palliative strategy. Current technologies should enable an improved selection of initial palliative approach through thoughtful, randomized trials.

Clinical characteristics and outcomes in patients with endocardial fibroelastosis associated with critical aortic stenosis with biventricular circulation

Clinical characteristics and outcomes in patients with endocardial fibroelastosis associated with critical aortic stenosis with biventricular circulation

Transcarotid balloon valvuloplasty in neonates and small infants with critical aortic valve stenosis utilizing continuous transesophageal echocardiographic guidance: A 22 year single center experience from the cath lab to the bedside.

Utilization of continuous transesophageal echocardiographic guidance (cTEE) during transcarotid balloon valvuloplasty (TCBV) in neonates and small infants with critical aortic valve stenosis (AS) allows for continuous hemodynamic assessment and improved outcomes. Preferred method of intervention for critical AS remains controversial due to conflicting results. Since 1992, 30 neonates and small infants with critical AS and adequate left ventricular (LV) volumes underwent TCBV with cTEE. Critical AS was defined as ductal dependent systemic circulation, LV systolic dysfunction, or an echo gradient≥100 mm Hg with evidence of hypoperfusion. The median age at intervention was 4 days (range 1-54 days). Nineteen (63%) patients required PGE1 and 25 (85%) had LV dysfunction. All procedures were performed with cTEE guidance. The initial 15 patients were performed in the cath lab whereas the subsequent 15 patients were performed at the bedside without fluoroscopy. The peak systolic gradient decreased from 70 to 24 mm Hg (P<0.001). Four (13%) early deaths were secondary to associated cardiac anomalies although one patient developed severe aortic valve insufficiency (AI) immediately post intervention. At discharge, two patients (8%) had ≥moderate AI. At a mean follow-up of 9 years (range: 2.2-20 years), there were 15 additional aortic valve interventions. Freedom from aortic valve reintervention at 10 years was 55% and actuarial survival rate at 10 and 15 years was 82%. Bedsides TCBV with cTEE guidance is effective palliation for neonates and small infants with critical AS and allows for continuous hemodynamic assessment without the use of ionizing radiation. Our early and late results appear comparable to surgical valvotomy.

Biventricular Badness: Rare Images of Ebstein Anomaly of the Tricuspid Valve in a Patient with Hypoplastic Left Heart Syndrome.

We report a rare case of hypoplastic left heart syndrome coexisting in a patient with Ebstein anomaly of the tricuspid valve, which has previously been described only in pathological studies. A fetal echocardiogram at 27-weeks gestation showed severe aortic stenosis with evolving hypoplastic left heart syndrome, significant endocardial fibroelastosis, a dysplastic tricuspid valve with moderate regurgitation, right atrial and ventricular dilation, and signs of fetal congestive heart failure. Due to inadequate left heart size, the patient was not a candidate for fetal intervention for critical aortic stenosis, and repeat studies showed progression of the lesion through the pregnancy. The infant was delivered at 36-weeks gestation with signs of hydrops, and a postnatal echocardiogram confirmed hypoplastic left heart syndrome as well as severe Ebstein anomaly of the tricuspid valve. The infant did not survive to intervention.

Efficacy and safety of percutaneous transluminal balloon dilation to prevent progression of banding site stenosis after bilateral pulmonary artery banding.

To investigate the efficacy and safety of percutaneous transluminal balloon dilation (PTBD) for the treatment of bilateral pulmonary artery banding (bil-PAB) site stenosis. Although bil-PAB is an alternative initial treatment for high-risk neonates with hypoplastic left heart syndrome (HLHS) or critical aortic stenosis (cAS), those patients often suffer from desaturation because of progressive stenosis of the bil-PAB sites during the interstage period. We retrospectively evaluated the efficacy and safety of 11 consecutive PTBD procedures performed between 2006 and 2012 to treat bil-PAB site stenosis in four high-risk infants (three females) with HLHS or cAS. PTBD was repeated twice in two patients and three times in one patient over intervals. The mean balloon diameter (BD) and BD-to-band circumference (BC) ratio were 3.1 ± 0.5 mm and 0.31 ± 0.06, respectively. After the procedures, the mean minimum lumen diameter was dilated significantly from 1.1 ± 0.1 mm to 1.7 ± 0.3 mm (P < 0.01), and the mean peripheral oxygen saturation increased significantly from 75 ± 8% to 85 ± 4% (P < 0.01). All patients reached the next stage operation involving the Norwood & bidirectional Glenn or Ross procedure, after growth. No complications such as band rupture occurred. For progressive stenosis of bil-PAB sites, PTBD using a balloon size that did not exceed the BC (BD around 30% of the BC) was an effective and safe procedure.

The new generation is coming. Percutaneous implantation of the fully repositionable Lotus® aortic valve prosthesis: the first Polish experience

Transcatheter aortic valve implantation (TAVI) is nowadays an accepted method of treatment for patients with symptomatic severe aortic stenosis who are inoperable or at very high risk of classic surgical aortic valve replacement. The Lotus valve system is a new generation TAVI device composed of a self-expanding stent prosthesis with implemented bovine pericardial leaflets, which is designed to facilitate repositioning, resheathing, and retrieval, even in the fully expanded and functioning position before the final release. In addition, the Lotus valve is surrounded by a flexible membrane to seal paravalvular gaps between the prosthesis and native valve. We present the first Polish experiences with the Lotus valve system. Due to its unique features, the Lotus valve may improve the prognosis in patients with inoperable or high risk critical aortic stenosis.

Low rate of prenatal diagnosis among neonates with critical aortic stenosis: insight into the natural history in utero

To better understand the natural history and spectrum of fetal aortic stenosis (AS), we aimed to (1) determine the prenatal diagnosis rate of neonates with critical AS and a biventricular (BV) outcome, and (2) describe the findings at fetal echocardiography in patients diagnosed prenatally. A multicenter, retrospective study was performed on neonates who presented with critical AS and who were discharged with a BV outcome from 2000 to 2013. The prenatal diagnosis rate was compared with that reported for hypoplastic left heart syndrome (HLHS). We reviewed fetal echocardiographic findings in patients who were diagnosed prenatally. In only 10 (8.5%) of 117 neonates with critical AS and a BV outcome was the diagnosis made prenatally, a rate significantly lower than that for HLHS in the contemporary era (82%; P < 0.0001). Of the 10 patients diagnosed prenatally, all had developed left ventricular dysfunction by a median gestational age of 33 (range, 28-35) weeks. When present, Doppler abnormalities such as retrograde flow in the aortic arch (n = 2), monophasic mitral inflow (n = 3) and left-to-right flow across the foramen ovale (n = 8) developed late in gestation (median 33 weeks). The prenatal diagnosis rate of critical AS and a BV outcome among neonates is very low, probably owing to a relatively normal four-chamber view in mid-gestation with development of significant obstruction in the third trimester. The natural history contrasts with that of severe mid-gestation AS with evolving HLHS and suggests that the gestational timing of development of significant AS has an important impact on subsequent left-heart growth in utero.

Транскатетерное лечение дегенеративного критического стеноза аортального клапана у больного с тяжелой сердечной недостаточностью и хроническим лимфолейкозом

Surgical aortic valve replacement is the standard therapy for severe aortic valve stenosis, however one third of patients are rejected because of high surgical risk. Under medical treatment alone these patients have a very poor prognosis with a high mortality rate. We present a case of 70-year-old male patient with degenerative symptomatic critical aortic stenosis and chronic lymphocytic leukemia. Due to recurrence of leukemia, the patient was denied conventional open heart surgery. Within few months of palliative chemotherapy decompensated aortic stenosis with severe congestive heart failure developed. Such therapeutic alternative as transcatheter aortic valve implantation (TAVI) emerged as a lifesaving procedure for the patient that allowed performing full-dose chemotherapy later. We provide a comprehensive review of current indications and contraindications for TAVI.

0535: Percutaneous valvuloplasty versus surgical valvulotomy in neonates with critical aortic stenosis

This study was to compare outcomes and prognosis of newborns with critical aortic valve stenosis (AVS) who underwent either percutaneous or surgical intervention as first therapeutic option. Neonates with diagnosis of critical AVS (defined as aortic flow dependent on ductus arteriosus patency) were included in the study, and divided in group I (percutaneous valvuloplasty) and group II (surgical valvulotomy). From 2000 to 2013, 23 cases (19 males) were analyzed: 9 in group I and 14 in II, aged 0 to 28 days at diagnosis (< 7 days of age: 78% of group I vs 50% in II, p= NS). Weight was > 2.5 kg in 100% of group II and 78% of group I. Symptoms of heart failure were more frequent in group I. Echocardiography showed: LVSF < 28% in 67% of group I vs 43% group II (NS), LV fibroelastosis in 88% of group I and 21% group II (p< 0.05), mitral regurgitation in 77% of group I and 7% group II (p< 0.05). Aortic valve anatomy included bicuspidia in 52% of cases, aortic tricuspide valve dysplasia in 43.5%. Analysis of events occurring within 36 months after first intervention showed: significant restenosis in 6 cases (26%= 55.5% in group I and 7% in group II), aortic valve regurgitation >grade2 in 11 cases (47.8%= 44% of group I and 50% of group II), Ross procedure performed in 5 cases (21.7%= 22.2% of group I and 21.4% of group II). Prognosis factors for reintervention were: aortic valve dysplasia, LV fibroelastosis and aortic annulus diameter <6 mm in group I (reintervention frequency respectively= 100%, 75% and 83%), and bicuspidia, aortic valve annulus <6 mm in group II (reintervention frequency respectively= 33% and 50%). Results of percutaneous valvuloplasty compared to surgical valvulotomy in neonates with AVS showed higher rates of reintervention in the percutaneous group and more severe AR in the surgical group. Aortic annulus diameter < 6 mm was related to reintervention risk.

Fetal cardiac interventions - are we ready for them?

The aim of the study was to analyze types and methods of intrauterine fetal cardiac interventions performed between June 2011 and December 2013, and to assess the perinatal management of the neonates. The program was developed after analysis of the available literature, practical individual training in Linz, Austria, and simulation of the procedure in a dissecting-room. The rules for anesthesia in pregnant women and their fetuses were developed. The interventions were performed in fetuses with critical cardiac defects, in the operating room, under ultrasonographic control. The protocol was approved by the Local Bioethics Committee at the Centre of Postgraduate Medical Education. We included fetuses with critical aortic stenosis (n=29), critical pulmonary stenosis (n=2), and closed or extremely restricted foramen ovale (n=7). Between June 2011 and December 2013, the team comprised of JD, MD and AK conducted 42 interventions in 35 fetuses, including 32 balloon aortic valvuloplasties (in 29 fetuses), 2 pulmonary valvuloplasties, 4 balloon atrial septostomies and 4 atrial septal stent placement. Three fetuses required both, aortic valvuloplasty and fenestration of the atrial septum. Out of the 42 procedures, 41 (97%) were technically successful. We recorded 3 cases of fetal demise associated with the intervention. We modulated the protocol of anesthesia given to pregnant women, switching from general to local anesthesia with intravenous sedation. We always provided additional fetal anesthesia with fentanyl and atracurium via the umbilical vein. Based on our 2.5-year experience, it seems safe to conclude that all types of fetal cardiac interventions may be successfully conducted at Polish centers. The procedures are safe for the pregnant women and improve fetal status. Most of the neonates treated prenatally were referred in good general condition to a tertiary pediatric cardiology and cardiac surgery center

Urgent Balloon Aortic Valvuloplasty for Worsening Heart Failure in a Patient with Acute Myocardial Infarction and Critical Aortic Stenosis.

A 91-year-old man was admitted to our hospital with dyspnea and chest pain. His electrocardiogram showed ST-segment elevation in the V1-4 leads, and an emergency coronary angiogram revealed subtotal occlusion in the left descending coronary artery. A successful primary percutaneous coronary intervention was subsequently performed using aspiration thrombectomy and bare metal stent implantation. However, his hemodynamic condition deteriorated with severe pulmonary congestion. Administration of inotropes with diuretics and mechanical ventilation were not effective, and an echocardiogram showed coexisting severe aortic stenosis. As a bailout procedure, we performed balloon aortic valvuloplasty (BAV) with a 12- and 14-mm balloon on the next day. After BAV, the patient's condition improved, and he was weaned from the ventilator. It is commonly understood that onset of acute myocardial infarction may trigger worsening of heart failure in patients with compensated aortic stenosis. However, emergency balloon aortic valvuloplasty, when timely administered, is considered a simple and effective procedure for such patients, and can lead to substantial clinical improvement. Acute coronary syndrome; Balloon aortic valvuloplasty; Congestive heart failure; Valvular heart disease.

Successful palliative valvuloplasty for critical aortic valve stenosis in aspartylglucosaminuria (AGU) syndrome

Successful palliative valvuloplasty for critical aortic valve stenosis in aspartylglucosaminuria (AGU) syndrome