The ascending aorta with bicuspid aortic valve: a phenotypic classification with potential prognostic significance.

OBJECTIVES: Different methods to classify the anatomical configurations of the aorta with bicuspid aortic valve (BAV) have been proposed. We aimed to test them in terms of descriptive power (i.e. capability to identify different clusters of patients with unique associations of anatomo-clinical features) and possible prognostic significance. METHODS: A consecutive echocardiographic series of 696 BAV patients (mean age 48 ± 16 years, male:female ratio 3:1) was analysed. Three possible schemes for classification of the patterns of aortic dimensions were compared. One defined the aortic shape as 'N' (ascending < sinuses > sinotubular junction (STJ)), 'A' (ascending > sinuses > STJ) or 'E' (sinotubular ≥ sinuses), the second as 'non-dilated', 'ascending phenotype' (dilated, with ascending > sinuses) or 'root phenotype' (dilated, with sinuses > ascending) and the third as normal, 'type I' (dilated only at the ascending tract), 'type II' (dilated at both ascending and sinus levels) or 'type III' (dilated only at the sinuses). We evaluated the correlation with valve morphotypes (right-left fusion, right-non-coronary fusion) and patient characteristics. In a smaller longitudinal study (n = 150), the occurrence of fast growth of the aorta (fifth quintile: ≥1 mm/year) during follow-up (5 ± 3 years) in the various phenotypes was assessed. RESULTS: The three classification methods proved meaningful in terms of association with valve morphotypes: significant associations were found between right-left-coronary BAV and the root phenotype (P < 0.001) and between the right-non-coronary BAV and the shapes A and E (P<0.001) as well as type I aortic configuration (P < 0.001). The aortic shape showed significant association with five of the other tested clinical variables, the phenotype and the type of dilatation with eight. In the longitudinal study, the root phenotype showed the most significant association with fast growth (>1 mm/year) of the ascending diameter (50% root phenotype patients; P = 0.005). The association with the N type was weaker (P = 0.055); no association was found with types from the other classification scheme (P = 0.42). CONCLUSIONS: When tested on a large population, three previously suggested phenotypic classifications of the BAV aorta proved to categorize patients into significantly different clusters, but only the classification system distinguishing between ascending phenotype and root phenotype showed a potential prognostic value. Phenotypic class of the aorta could be a factor to integrate in future comprehensive models for risk stratification of BAV aortopathy.

Towards an individualized approach to bicuspid aortopathy: different valve types have unique determinants of aortic dilatation.

OBJECTIVES: Bicuspid aortic valve (BAV)-related aortopathy is increasingly recognized to be a heterogeneous disease entity, although the surgical approach, from indications to techniques, is still standard rather than individualized. We aimed to define the determinants of aortic dilatation in BAV patients stratified according to the valve morphotype. METHODS: A consecutive echocardiographic series of 622 BAV patients was analysed. Among demographic (age, sex), anthropometric (height, weight, body surface area, body mass index), clinical (associated diseases) and echocardiographic variables (valve function, ventricular parameters), the determinants of aortic root and ascending tract diameter were assessed by multivariate regression models, as well as the predictors of aortic dilatation (size index >2.1 cm/m(2)) both in the overall population and separately in groups of different valve morphotypes (RL, right-left fusion; RN, right-non-coronary fusion). RESULTS: Independent determinants of aortic root diameter (at sinuses) were age (P < 0.001), significant aortic regurgitation (P < 0.001), sex (female protective, P < 0.001) and valve morphotype (RN protective, P < 0.001). Independent determinants of ascending aortic diameter (tubular tract) were age (P < 0.001), RN morphotype (P < 0.001), body mass index (P = 0.005) and chronic obstructive pulmonary disease (P < 0.001). In univariate analysis, the RL morphotype was associated with dilatation (ASI > 2.1 cm/m(2)) at sinuses in 41% cases vs 22% for RN (P < 0.001), and the RN morphotype was associated with dilatation at the tubular tract in 68 vs 56% for RL (P = 0.007). The presence of root dilatation was predicted by age and absence of significant stenosis in the RL morphotype subgroup, and by severe regurgitation in the RN subgroup. In the RL-type subgroup, non-regurgitant aortic valve and chronic lung disease predicted dilatation at the ascending level; and in the RN-type subgroup, age and obesity. CONCLUSIONS: The two most common BAV morphotypes are associated with aortic dilatation at two different tracts (RL at the root; RN at the tubular ascending tract) independently of valve function. Moreover, the determinants of aortic dilatation were at least in part different between the two morphotypes: this may provide stratification criteria for individualized methods of follow-up and treatment.

Pattern of ascending aortic dimensions predicts the growth rate of the aorta in patients with bicuspid aortic valve.

OBJECTIVES: This study sought to identify risk factors for rapid growth of the ascending aorta in patients with bicuspid aortic valve (BAV) disease, taking into account its phenotypic variability. BACKGROUND: Phenotypic heterogeneity of BAV-related aortopathy has recently been widely recognized. However, few studies have addressed the determinants of aortic growth so far, not distinguishing among morphological phenotypes. METHODS: Serial retrospective data on 133 adult outpatients with BAV undergoing echocardiographic follow-up were analyzed to search for factors associated with aortic diameter growth over time and with rapid aortic growth (fifth quintile of growth rate distribution), focusing on the impact of different valve morphotypes (i.e., cusp fusion pattern: right-left coronary [RL] and right-noncoronary [RN]) and previously defined aortic phenotypes (nondilated aorta, ascending dilation, root dilation). RESULTS: The RL pattern was present in 69% of patients with BAV and RN in 31%. At baseline, an ascending dilation phenotype was observed in 57% of patients and a root phenotype in 13.5%. No patient with RN-BAV had a root dilation phenotype at either baseline or last examination. Follow-up time averaged 4.0 ± 2.7 years (535 patient-years). The mean growth rate was 0.3 mm/year at the sinuses and 0.6 mm/year at the ascending level. Aortic regurgitation predicted an increase in ascending diameter over time (odds ratio [OR]: 2.3; p = 0.03). Root phenotype at presentation, not absolute baseline diameter, was an independent predictor of fast progression (>0.9 mm/year) for the ascending tract (OR: 14; p = 0.001). Fast growth was rarely seen in patients with the RL morphotype and ascending phenotype (6% at the root and 10% at the ascending level). CONCLUSIONS: In patients with BAV, the root phenotype (aortic dilation predominantly at the sinuses, with normal or less dilated ascending tract) may be a marker of more severe aortopathy, warranting closer surveillance and earlier treatment. The more common ascending phenotype proved to be a more stable disease entity, generally with slower progression.

Primary cardiac angiosarcoma in a 25-year-old man: excision, adjuvant chemotherapy, and multikinase inhibitor therapy.

Primary cardiac tumors do not occur frequently, and only one quarter of them, chiefly sarcomas, are malignant. Patients with angiosarcoma typically have a shorter survival time than do patients with other sarcomas, and the prognosis for survival depends strictly on the stage of the disease at the time of diagnosis and the possibility of complete surgical excision. Chemotherapy and radiotherapy have well-established postoperative roles because of the high probability of metastasis. We report the case of a 25-year-old man who presented with pericardial effusion and echocardiographic evidence of an intracavitary right atrial mass but without the bulky, infiltrative growth typical of this location of the disease. Malignancy was suggested by the clinical presentation, the location of the mass in the right side of the heart, and the absence of conditions favoring thrombus formation. After complete surgical excision, the mass was confirmed to be an angiosarcoma. Conventional adjuvant chemotherapy and maintenance therapy with inhibitors of CD117 (c-kit) and vascular endothelial growth factor relieved the patient's clinical symptoms and enabled his long-term, disease-free survival. In addition to reporting this case, we discuss aspects of the diagnosis and treatment of angiosarcoma.

Endovascular stent-graft treatment of thoracic aortic syndromes: a 7-year experience.

Thoracic aortic diseases (TAD) are relatively frequent conditions associated with high mortality. Recently, several reports have demonstrated the safety and efficacy of endovascular stent-graft (EVG) placement for TAD as an alternative to open surgery. We report our experience in management of thoracic aortic syndrome on 56 consecutive patients with TAD that underwent endovascular stent-graft repair. MDCT angiography was used in all patients to provide preprocedure evaluation and measurements. In particular it is necessary to evaluate the proximal and distal landing zones of the stent-graft. All EVGs in our series were placed successfully. Conversion to open surgery was never required. Six patients (10.7%) died early after the stent-graft deployment. During follow-up four more patients died. The endoleak rate was 16.7% (no. 10 pt). We did not observe any case of paraplegia. The present study shows the efficacy of EVG in the long-term follow-up, with an overall survival of 82.1%, which is comparable to that reported in recent studies. In conclusion this technique is emerging as an alternative approach in the treatment of TAD because this approach offers a less invasive therapeutic option to standard surgical techniques, even in patients who have associated diseases that make them poor surgical candidates.

"Z-plasty suture": a new procedure for complex reconstructions of posterior mitral leaflet.

In recent years the conservative techniques to treat degenerative mitral valve insufficiency have developed to such an extent mainly due to a better understanding of the physiology and pathology of the mitral valve and to the possibility to get predictable and satisfactory results. Still a challenge persists for the cardiac surgeon when he has to deal with complex reconstructions. The technique described seems to offer an even better surgical option for patients with complex lesions involving the posterior mitral leaflet, especially as far as the hemodynamic performance is concerned.

Predictors of ascending aortic dilatation with bicuspid aortic valve: a wide spectrum of disease expression.

OBJECTIVE: This study aimed to describe the features and identify the predictors of ascending aorta dilatation in patients with congenital bicuspid aortic valve (BAV). METHODS: In 280 adult patients with isolated BAV undergoing echocardiography, multivariate logistic regression models, including clinical and echocardiographic variables, were developed to predict dilatation (aortic ratio exceeding 1.1) at both ascending and root level. Factors predicting aneurysm with surgical indication were also investigated. Classification tree models were used to identify factors influencing the probability of having a small aorta, normal aortic dimensions, a dilated ascending aorta or a dilated root (aortic phenotypes). RESULTS: Aortic dilatation was present in 83.2% patients, prevailing at the mid-ascending tract in 83.7% of them. Surgical indication criteria were reached in 43.2% patients. A small aortic root was found in 16 patients (5.7%), thereafter excluded from multivariate models predicting dilatation. Age (maximal risk at 50-60 years: OR=13.7; reference category: <30 years) and severe aortic stenosis (OR=23.8) independently predicted mid-ascending dilatation (p<0.001). Male gender (OR=4.1, p=0.001), age >60 (OR=2.6, p=0.022) and severe regurgitation (OR=3.9, p=0.011) were determinants of root involvement, while stenosis (> or =moderate; OR=0.3, p<0.001) was a protective factor. Aortic stenosis (any degree, OR=2.4) and hypertension (OR=4.3) were the most significant predictors of mid-ascending aneurysm reaching surgical indication. Classification analysis showed that increasing age significantly increased the prevalence of ascending dilation phenotype, stenosis increased the prevalence of small aorta phenotype, and male gender of root dilation phenotype. Once excluding patients with small aortas from the analysis, a positive correlation was observed between degree of stenosis and mid-ascending size (p=0.016). CONCLUSIONS: BAV patients constitute an importantly heterogeneous population in terms of risk and features of aortic disease. The most common condition is an ectasia of the mid-ascending tract, with unaffected or mildly involved root. If further confirmed, this could suggest that surgical approach may spare the root in most BAV patients. Mid-ascending dilatation is proportional to stenosis severity, suggesting a post-stenotic causative mechanism. Root dilatation is rarer, mostly observed in younger men, and unrelated to the presence and severity of stenosis. The two different aortic dilatation phenotypes (mid-ascending and root) may be subtended by different pathogeneses.

A rare complication of atrial septal occluders: diagnosis by transthoracic echocardiography.

Atrial septal defect transcatheter occlusion techniques have become a reliable alternative to surgical procedures. Possible complications can derive from unfavorable anatomy of the defect and over-dimensioning of the device. We describe the first case ever reported of anterior mitral leaflet perforation caused by an atrial septal occluder. The diagnosis was performed by conventional echocardiography and 3 dimensions helped for a more accurate anatomic definition. Device removal, atrial septal defect closure, and repair of the mitral tear were then successfully performed.

Echocardiographic anatomy of ascending aorta dilatation: correlations with aortic valve morphology and function.

BACKGROUND: Different anatomical forms of proximal aortic dilations associated with aortic valve disease can be distinguished by echocardiography. Differences in the anatomy could reflect different pathogeneses and need for different therapeutic approaches. The present study assessed the clinical features associated to each anatomical form, particularly focusing on the relations with valve morphology and function. METHODS: Trans-thoracic and trans-esophageal echocardiography reports of 552 adult patients (mean age 60.4+/-12.8 years; 379 male) with mild to severe proximal aorta dilation were reviewed. The relationships between the anatomy of aorta dilatation (distinguished into "root type" dilatation, with maximal enlargement at the sinuses, and "mid-ascending type", with maximal diameter at the mid-ascending tract) and aortic valve morphology (tricuspid/bicuspid) and function (normal/stenosis/regurgitation) were assessed. The relations with other clinico-echocardiographic variables were also tested in univariate and multivariate analysis. RESULTS: A "root type" dilatation was found in 4.9% tricuspid patients with stenosis, 32.3% with regurgitation, 22.5% with normal valve function (p=0.018). Dilatation prevailed at the mid-ascending tract in patients with bicuspid aortic valve, irrespective of valve function (stenotic: 92.9%, regurgitant: 87.9%, normal: 94.3%; p=0.23). Predominant root involvement was significantly more prevalent in male patients (24.8% versus 5.2% in females; p<0.001). In multivariate analysis, predominant aortic valve regurgitation (OR=1.83; p=0.028) independently predicted root site, while predominant aortic valve stenosis (OR=3.70; p=0.001), bicuspidity (OR=2.90; p=0.005) and female sex (OR=6.10; p<0.001) predicted mid-ascending site. CONCLUSIONS: Pathogenetical considerations arise from the evidence of preferential mid-ascending localization of bicuspid-associated aortic dilatations. This finding is consistent with previous studies on bicuspid valve models revealing a wall stress overload beyond the sino-tubular ridge.

Treatment of type B aortic dissection: endoluminal repair or conventional medical therapy?

OBJECTIVE: To evaluate the mid-term results of endovascular stent-grafting for type B aortic dissection, in comparison with those of standard medical therapy in uncomplicated cases. METHODS: Between January 1999 and 2004, among 56 patients (mean age 59.5+/-11.5 years) with type B aortic dissection, hypotensive medical therapy was the only treatment in 28 uncomplicated cases, (group A), while stent-graft implantation was performed in 28 patients with uncontrolled hypertension, persistent pain or evidence of dissection progression or complication (group B). In 14 cases (50%) the procedure was performed in an acute setting. Stent-grafting procedures were monitored with intraoperative trans-esophageal echocardiography and cine-angiography. CT scan and trans-esophageal echocardiography were performed before hospital discharge, at 6 and 12 months and then yearly. RESULTS: Follow-up (range 1-61 months, average 18.1+/-16.9 months) was 100% complete. In-hospital mortality was 10.7% (three patients, all belonging to Group B; P=0.24). No spinal cord injuries were observed. Early endoleak occurred in one patient (3.5%). Mid-term mortality was lower in Group B, although the difference was not significant (10.7 versus 14.3% in Group A, P=0.71). Follow-up CT scans evidenced complete thrombosis of the false lumen in 75% cases in Group B, 10.7% in Group A (P=0.0001), and an aneurismal dilatation of the descending aorta in 3.5% cases in Group B, 28.5% in Group A (P=0.02). CONCLUSIONS: Although with still considerable early mortality, endovascular stent-graft implantation is an effective option for the treatment of complicated type B aortic dissection. Endovascular treatment achieved a better mid-term fate of the descending thoracic aorta than medical therapy alone, even in patients with worse preoperative conditions.

Asymmetric medial degeneration of the ascending aorta in aortic valve disease: a pilot study of surgical management.

BACKGROUND AND AIM OF THE STUDY: In structural aortic valve disease, medial degeneration is often associated with ascending aorta dilatation. This pathology is often asymmetric, with more severe involvement of the convex aspect of the ascending aorta. The condition can be managed with surgical excision of the diseased portion of the aortic wall and remodeling reconstruction at the time of aortic valve replacement (AVR). METHODS: Sixty-one patients underwent AVR and 'waistcoat aortoplasty'. Sinus dilatation was repaired by plicating the subcoronary redundant aortic wall using anchoring sutures of the valve prosthesis. The diseased aortic wall was removed via a triangular resection, and the aorta reconstructed with a double-layer technique, to achieve autologous reinforcement of the convex right posterolateral wall and stress reduction on the aortotomy suture line. Echocardiography was performed preoperatively, immediately postoperatively, and every six months thereafter. The significance of diameter variations was evaluated. RESULTS: There was no hospital mortality, and only low postoperative morbidity. Post-reduction diameters at the sinuses, sinotubular junction and ascending aorta were significantly less than preoperative values (p < 0.0001). During a mean follow up of 33.8 +/- 10.2 months (range: 13-50 months), no significant increase in root (p = 0.32), sinotubular (p = 0.15), or ascending diameter (p = 0.11) was observed. CONCLUSION: The 'waistcoat aortoplasty' should be considered when dealing with asymmetric ascending dilation secondary to aortic valve disease-related medial degeneration. However, additional studies with a longer follow up are needed to confirm these findings.

Unusual cases of infective endocarditis.

We report 2 patients with unusual cases of infective endocarditis. The first patient had a large, mural vegetation on left ventricle that was diagnosed with transthoracic echocardiography; and the second patient had a large, mobile vegetation in the descending prosthetic aorta with an abscess cavity around the vessel, diagnosed by transesophageal echocardiography. This report confirms the usefulness of transthoracic and transesophageal echocardiography in the diagnosis and management of uncommon cases of endocarditis.