Association of pulmonary atresia with intact ventricular septum and aortic valve stenosis. Prenatal diagnosis

A rare association of pulmonary atresia with an intact septum was diagnosed through echocardiography in a fetus 32 weeks of gestational age. The diagnosis was later confirmed by echocardiography of the newborn infant and further on autopsy. The aortic valve was bicuspid with a pressure gradient of 81mmHg, and the right ventricle was hypoplastic, as were the pulmonary trunk and arteries, and the blood flow was totally dependent on the ductus arteriosus.

Performance of Surgical Risk Scores to Predict Mortality after Transcatheter Aortic Valve Implantation

AbstractBackground:Predicting mortality in patients undergoing transcatheter aortic valve implantation (TAVI) remains a challenge.Objectives:To evaluate the performance of 5 risk scores for cardiac surgery in predicting the 30-day mortality among patients of the Brazilian Registry of TAVI.Methods:The Brazilian Multicenter Registry prospectively enrolled 418 patients undergoing TAVI in 18 centers between 2008 and 2013. The 30-day mortality risk was calculated using the following surgical scores: the logistic EuroSCORE I (ESI), EuroSCORE II (ESII), Society of Thoracic Surgeons (STS) score, Ambler score (AS) and Guaragna score (GS). The performance of the risk scores was evaluated in terms of their calibration (Hosmer–Lemeshow test) and discrimination [area under the receiver–operating characteristic curve (AUC)].Results:The mean age was 81.5 ± 7.7 years. The CoreValve (Medtronic) was used in 86.1% of the cohort, and the transfemoral approach was used in 96.2%. The observed 30-day mortality was 9.1%. The 30-day mortality predicted by the scores was as follows: ESI, 20.2 ± 13.8%; ESII, 6.5 ± 13.8%; STS score, 14.7 ± 4.4%; AS, 7.0 ± 3.8%; GS, 17.3 ± 10.8%. Using AUC, none of the tested scores could accurately predict the 30-day mortality. AUC for the scores was as follows: 0.58 [95% confidence interval (CI): 0.49 to 0.68, p = 0.09] for ESI; 0.54 (95% CI: 0.44 to 0.64, p = 0.42) for ESII; 0.57 (95% CI: 0.47 to 0.67, p = 0.16) for AS; 0.48 (95% IC: 0.38 to 0.57, p = 0.68) for STS score; and 0.52 (95% CI: 0.42 to 0.62, p = 0.64) for GS. The Hosmer–Lemeshow test indicated acceptable calibration for all scores (p > 0.05).Conclusions:In this real world Brazilian registry, the surgical risk scores were inaccurate in predicting mortality after TAVI. Risk models specifically developed for TAVI are required.

Transcatheter Aortic Valve Implantation and Morbidity and Mortality-Related Factors: a 5-Year Experience in Brazil

Abstract Background: Transcatheter aortic valve implantation has become an option for high-surgical-risk patients with aortic valve disease. Objective: To evaluate the in-hospital and one-year follow-up outcomes of transcatheter aortic valve implantation. Methods: Prospective cohort study of transcatheter aortic valve implantation cases from July 2009 to February 2015. Analysis of clinical and procedural variables, correlating them with in-hospital and one-year mortality. Results: A total of 136 patients with a mean age of 83 years (80-87) underwent heart valve implantation; of these, 49% were women, 131 (96.3%) had aortic stenosis, one (0.7%) had aortic regurgitation and four (2.9%) had prosthetic valve dysfunction. NYHA functional class was III or IV in 129 cases (94.8%). The baseline orifice area was 0.67 ± 0.17 cm2 and the mean left ventricular-aortic pressure gradient was 47.3±18.2 mmHg, with an STS score of 9.3% (4.8%-22.3%). The prostheses implanted were self-expanding in 97% of cases. Perioperative mortality was 1.5%; 30-day mortality, 5.9%; in-hospital mortality, 8.1%; and one-year mortality, 15.5%. Blood transfusion (relative risk of 54; p = 0.0003) and pulmonary arterial hypertension (relative risk of 5.3; p = 0.036) were predictive of in-hospital mortality. Peak C-reactive protein (relative risk of 1.8; p = 0.013) and blood transfusion (relative risk of 8.3; p = 0.0009) were predictive of 1-year mortality. At 30 days, 97% of patients were in NYHA functional class I/II; at one year, this figure reached 96%. Conclusion: Transcatheter aortic valve implantation was performed with a high success rate and low mortality. Blood transfusion was associated with higher in-hospital and one-year mortality. Peak C-reactive protein was associated with one-year mortality.

Short- and midterm follow-up results of valvuloplasty with balloon catheter for congenital aortic stenosis

OBJECTIVE: To report short and midtem follow-up results of balloon aortic valvuloplasty to treat congenital aortic stenosis. METHODS: Seventy-five patients (median age: 8 years) underwent the procedure through the retrograde femoral or carotid route. RESULTS: The procedure was completed in 74 patients (98.6%). The peak-to-peak systolic gradient dropped from 79.6±27.7 to 22.3±17.8 mmHg (P<0.001), the left ventricular systolic pressure dropped from 164±39.1 to 110±24.8 mmHg (P<0.001), and the left ventricular end diastolic pressure dropped from 13.3±5.5 to 8.5±8.3 mmHg (P< 0.01). Four patients (5.3%) died due to the procedure. Aortic regurgitation (AoR) appeared or worsened in 27/71 (38%) patients, and no immediate surgical intervention was required. A mean follow-up of 50±38 months was obtained in 37 patients. Restenosis and significant AoR were observed in 16.6% of the patients. The estimates for being restenosis-free and for having significant AoR in 90 months were 60% and 50%, respectively. CONCLUSION: Aortic valvuloplasty was considered the initial palliative method of choice in managing congenital aortic stenosis, with satisfactory short- and midterm results.

Unsupported valvuloplasty in children with congenital mitral valve anomalies. Late clinical results

OBJECTIVE: To analyze late clinical evolution after surgical treatment of children, with reparative and reconstructive techniques without annular support. METHODS: We evaluated 21 patients operated upon between 1975 and 1998. Age 4.67±3.44 years; 47.6% girls; mitral insufficiency 57.1% (12 cases), stenosis 28.6% (6 cases), and double lesion 14.3% (3 cases). The perfusion 43.10±9.50min, and ischemia time were 29.40±10.50min. The average clinical follow-up in mitral insufficiency was 41.52±53.61 months. In the stenosis group (4 patients) was 46.39±32.02 months, and in the double lesion group (3 patients), 39.41±37.5 months. The echocardiographic follow-up was in mitral insufficiency 37.17±39.51 months, stenosis 42.61±30.59 months, and in the double lesion 39.41±37.51 months. RESULTS: Operative mortality was 9.5% (2 cases). No late deaths occurred. In the group with mitral insufficiency, 10 (83.3%) patients were asymptomatic (p=0.04). The majorit y with mild reflux (p=0.002). In the follow-up of the stenosis group, all were in functional class I (NYHA); and the mean transvalve gradient varied between 8 and 12mmHg, average of 10.7mmHg. In the double lesion group, 1 patient was reoperated at 43 months. No endocarditis or thromboembolism were reported. CONCLUSION: Mitral stenosis repair has worse late results, related to the valve abnormalities and associated lesions. The correction of mitral insufficiency without annular support showed good long-term results.

Protective Effect of Aortic Stenosis on the Coronary Arteries. Hypothetic Considerations to an Old Enigma

Abstract A literature overview of angiographic studies has shown that the prevalence of significant coronary disease in patients with aortic stenosis (AS) varies from 20 to 60%. Early necropsy studies suggested that patients with AS had a lower than expected incidence of coronary artery disease (CAD), originating the concept of a protective effect of AS on the coronary arteries. The myth of AS protection against CAD would be better explained as endothelium-myocardial interaction (crosstalk) protection triggered by left ventricular overload. Therefore, the cGMP/NO pathway induced by the AS overload pressure would explain the low incidence of CAD, which is compatible with the amazing natural long-term evolution of this cardiac valve disease.

Mycoplasma pneumoniae and Chlamydia pneumoniae in calcified nodules of aortic stenotic valves

Aortic Valve Stenosis (AVS) has been explained as an atherosclerotic process of the valve as they often exhibit inflammatory changes with infiltration of macrophages, T lymphocytes and lipid infiltration. The present study investigated whether the bacteria Chlamydia pneumoniae (CP) and Mycoplasma pneumoniae (MP), detected previously in atherosclerotic plaques, are also present in AVS. Ten valves surgically removed from patients with AVS were analyzed by immunohistochemistry, in situ hybridization, and electron microscopy. The mean and standard deviation of the percentage areas occupied by CP antigens and MP - DNA were respectively 6.21 +/- 5.41 and 2.27 +/- 2.06 in calcified foci; 2.8 +/- 3.33 and 1.78+/- 3.63 in surrounding fibrotic areas, and 0.21 +/- 0.17 and 0.12 +/- 0.13 in less injured parts of the valve. There was higher amount of CP and MP in the calcified foci and in the surrounded fibrosis than in more preserved valvular regions. In conclusion, the fact that there were greater amounts of CP and MP in calcification foci of AVS favors the hypothesis that AS is not an inevitable degenerative process due to aging, but rather that it may be a response to the presence of these bacteria, similarly to the morphology detected in atherosclerosis damage.

Aortic valve assessment. Anatomical study of 100 healthy human hearts

PURPOSE: To assess anatomical characteristics of the aortic valve, so that they may be useful in diagnostic situations and surgical treatment. METHODS: The study analyzed 100 healthy fixed human hearts; 84% of them obtained from males, 61% of them from Caucasian individuals. The ages of the individuals ranged from 9 to 86 years (mean 30±15.5 years). The characteristics assessed related to age, sex, and race were the following: number and height of the cusps, size of the lunulae, internal and external intercommissural distance, position of the coronary ostium in relation to the aortic valve, position of the ventricular septum in relation to the aortic valve, thickness of the cusps. RESULTS: All hearts assessed had a tricuspidal aortic valve. In regard to the height of the cusps and size of the lunula, the left coronary cusp was larger, followed by the right coronary cusp and the noncoronary cusp. The internal and external intercommissural distances had mean values of 24.6±5.7mm and 19.7±7mm, respectively. In regard to the position of the coronary ostia, in one heart two ostia emerged from the left coronary sinus, and in another, the ostium was supracommissural. The mean diameter of the aorta was 21.8±3.6mm, and there were no significant sexual or racial differences, but the diameter increased progressively with the increase in age. The thickness of the cusps did not show any significant difference in the 3 points assessed. CONCLUSION: The aortic valve annulus did not show a perfect circumference, with some variations in the measurements of the annulus, in the cusps and in the relation with the ventricular septum.

Absence of the aortic valve associated with hypoplastic left-sided heart syndrome

In this report we describe the twelveth case in the literature of absence of the aortic valve cusps, associated with hypoplastic left-sided heart syndrome in a neonate. Clinical and hemodynamic conditions in our patient resemble the classical features of this syndrome except for a greater development of the ascending aorta and the left ventricular cavity, due to aortic insufficiency. A patch was unsuccessfully inserted at the aortic annulus to exclude the left ventricle from the circulation. In addition the Norwood operation was performed.