Reliability and identification of aortic valve prolapse in the horse.

BACKGROUND: The objectives were to determine and assess the reliability of criteria for identification of aortic valve prolapse (AVP) using echocardiography in the horse. RESULTS: Opinion of equine cardiologists indicated that a long-axis view of the aortic valve (AoV) was most commonly used for identification of AVP (46%; n=13). There was consensus that AVP could be mimicked by ultrasound probe malignment. This was confirmed in 7 healthy horses, where the appearance of AVP could be induced by malalignment. In a study of a further 8 healthy horses (5 with AVP) examined daily for 5 days, by two echocardiographers standardized imaging guidelines gave good to excellent agreement for the assessment of AVP (kappa>0.80) and good agreement between days and observers (kappa >0.6). The technique allowed for assessment of the degree of prolapse and measurement of the prolapse distance that provided excellent agreement between echocardiographers, days and observers (kappa/ICC>0.8). Assessments made using real-time zoomed images provided similar measurements to the standard views (ICC=0.9), with agreement for the identification of AVP (kappa>0.8). Short axis views of the AoV were used for identification of AVP by fewer respondents (23%), however provided less agreement for the identification of AVP (kappa>0.6) and only adequate agreement with observations made in long axis (kappa>0.5), with AVP being identified more often in short axis (92%) compared to long axis (76%). Orthogonal views were used by 31% of respondents to identify the presence of AVP, and 85% to identify cusp. Its identification on both views on 4 days was used to categorise horses as having AVP, providing a positive predictive value of 79% and negative predictive value of 18%. Only the non-coronary cusp (NCC) of the AoV was observed to prolapse in these studies. Prolapse of the NCC was confirmed during the optimisation study using four-dimensional echocardiography, which concurred with the findings of two-dimensional echocardiography. CONCLUSIONS: This study has demonstrated reliable diagnostic criteria for the identification and assessment of AVP that can be used for longitudinal research studies to better define the prevalence and natural history of this condition.

Comparison of magnetic resonance imaging with transthoracic echocardiography in the diagnosis of ventricular septal defect-associated coronary cusp prolapse.

PURPOSE: To compare magnetic resonance imaging (MRI) with transthoracic echocardiography (TTE) in the diagnosis of coronary cusp prolapse (CCP) as a complication of ventricular septal defect (VSD). MATERIALS AND METHODS: Twenty-three patients (10 males, 13 females, mean age 9 years) with clinically suspected CCP were included in a retrospective study. Long and short axis cine images and electrocardiogram (ECG)-gated axial T1-weighted image MRI images were evaluated for deformity and protrusion of right and noncoronary cusps. We compared MRI and TTE results. We compared the agreement between MRI and TTE in outlet VSD and membranous VSD. RESULTS: On MRI, 19 patients had right coronary cusp prolapse (RCCP), three had noncoronary cusp prolapse (NCCP), and one patient had RCCP and NCCP on MRI. Twenty-two patients had RCCP on TTE and none had NCCP. Seventeen patients had outlet defects and six patients had a membranous defect at operation. All patients with an outlet VSD had RCCP on both MRI and TTE. The agreement between MRI and TTE in outlet VSD was better than in membranous VSD. CONCLUSION: MRI results corresponded with TTE results in patients with outlet VSDs, but discordant results were observed in patients with membranous VSDs.

Pulmonary vascular protective mechanisms in adult patients with an isolated large ventricular septal defect: a 21-year experience.

BACKGROUND: Ventricular septal defects (VSDs) are one of the most common congenital heart defects in adults. In adult patients with an anatomically large VSD and relatively preserved pulmonary vascular system, several pulmonary flow-limiting cardiac morphologic alterations (PFMA) are encountered. PATIENTS: Ninety-eight male patients (mean age 22.5 +/- 2 years) operated for an anatomically large VSD in our institution were retrospectively reviewed. PFMA in patients with an anatomically large but functionally mild-to-moderate VSD (when ratio of pulmonary to systemic flow (Q(p)/Q(s)) < 2.2 and ratio of pulmonary to systemic vascular resistance (R(p)/R(s)) < 0.3) were recorded. RESULTS: Thirty patients (31.2%) revealed a mild-to-moderate VSD in functional severity. Five PFMA were encountered in these patients: (1) ostium (os) infundibulum (n = 10, 33.3%), (2) aneurysm of the membranous septum (AMS) (n = 10, 33.3%), (3) systolic bulging of the conal septum toward the right ventricular outflow tract (n = 6, 20%), (4) prolapse of the aortic cusps (n = 2, 6.7%), and (5) attachment of the tricuspid septal leaflet to the septal crest (n = 2, 6.7%). Double-chambered right ventricle was encountered in four patients with os infundibulum and classic tetralogy-type septal malalignment in one patient with aortic cusp prolapse. Concurrent to VSD repair, resection of the os infundibulum and the AMS and aortic valve repair were performed. CONCLUSION: Presence of a large VSD and relatively preserved pulmonary vascular system in adults is associated with several PFMA. Preoperative awareness and concurrent surgical treatment of these alterations seem to be crucial to improve the expected benefit of surgical repair of VSD in this subgroup of the patients.

Transcatheter closure of ventricular septal defect in aortic valve prolapse and aortic regurgitation.

OBJECTIVE: To report intermediate follow-up result of transcatheter closure of ventricular septal defect (VSD) in presence of aortic valve prolapse (AVP) with or without aortic regurgitation (AR). METHOD: This is a retrospective review of 19 patients with VSD with AVP with AR who underwent transcatheter closure in between September 2011-July 2014. Mean age was 8 years (1-16 years, standard deviation [SD] 4.08 years) and mean weight was 26.03kg (9-81.5kg, SD 16.57kg). Among them 2 had subarterial VSD, 6 had subaortic VSD and 11 had perimembranous VSD. All of them had mild AVP and 13 of them had AR (trivial or mild). Median VSD size was 4.3mm (4-6mm). Transcatheter closure was done either by retrograde technique using the Amplatzer Duct Occluder-II in 17 patients or antegrade technique using the Duct Occluder-I in 2 cases. Mean follow-up period was 18 months (12-36 months). RESULT: Immediate major complications were encountered in 2 (10.5%) cases. Significant aggravation of device related AR was seen in one case & device embolised to right pulmonary artery in another case and both of them were managed surgically. During follow up, 1 child had significant additional VSD requiring device closure. One child developed moderate AR, requiring surgery. None of the other had shown any increase in severity of AR. CONCLUSION: Device closure of VSD in presence of mild AVP and mild AR appears to be safe. Longer follow-up is necessary to draw final conclusion.

Abbreviated aortic insufficiency in aortic dissection caused by prolapse of the intimal flap.

Aortic insufficiency was identified in a patient with acute ascending aortic dissection. The aortic insufficiency was limited to the first half of diastole by prolapse of the intimal flap against the regurgitant orifice. This unusual pathophysiology was well demonstrated by two-dimensional and color flow Doppler echocardiography.

An adult case with multiple cardiac valve prolapse and regurgitation.

A 56-year-old female had pure regurgitation in all cardiac valves. Color Doppler echocardiography showed a regurgitant jet in all cardiac valves. The severity of regurgitation due to the prolapse in all valves was moderate. The patient had no history of rheumatic fever, ischemic heart disease, endocarditis or hypertension. Physical characteristics of the patient were neither of Marfan's nor Ehlers-Danlos' syndrome. The etiology of regurgitation in all cardiac valves of this patient may be due to multiple valve prolapse.

[Aortic insufficiency in an asymptomatic girl: valve prolapse as the only finding]. / Insuficiencia aórtica en niña asintomática: prolapso valvular como único hallazgo.

A case of an asymptomatic 9-year-old girl is reported. She was diagnosed of aortic regurgitation by valvular prolapse without other systemic or valvular pathology associated. We discuss her etiology and physiopathology.

[Prevalence of tricuspid and aortic valve prolapse in patients with mitral valve prolapse]. / Prevalência de prolapso valvar tricúspide e aórtico em portadores de prolapso valvar mitral.

Two-dimensional echocardiograms were performed in 30 patients with mitral valve prolapse (15 females and 15 males, with an average of 33.3). The main objective was to observe the prevalence of involvement of tricuspid and aortic valves. Tricuspid valve prolapse was observed in 43.3% with anterior and septal involvement in 92.3% and posterior involvement in 15.3%. The incidence of aortic prolapse was 10% with involvement of both right coronary and non-coronary leaflets. All patients with aortic valve prolapse showed involvement of both mitral leaflets and at least two tricuspid leaflets. It is concluded that involvement of other valves such as tricuspid (43.3) and aortic (10%) is a common finding in patients with mitral valve prolapse.

[Valvular prolapse (clinical, echocardiographic, phonocardiographic and electrocardiographic features)]. / Prolapsy klapanov (klinicheskaia, ékhokardiograficheskaia, fonokardiograficheskaia i élektrokardiograficheskaia kharakteristiki).

Clinical, echoCG, PCG and ECG examinations were performed in 42 patients with primary isolated valve prolapses: mitral, aortal and tricuspid (32, 5 and 5 patients, respectively). It is shown that degree 1 regurgitation caused no hemodynamic disturbances, that of degree 2 and 3 brought about cardial complaints in more than half the patients, hemodynamic overstrain of the heart, sound symptoms similar to those of relevant valvular disease.

Aorta-to-right ventricular outflow tract fistula with coronary cusp prolapse.

We describe a rare case of aorta-to-right ventricular fistula in a 20-year-old man who presented with signs and symptoms of congestive heart failure. He was successfully treated by closing the fistula with a Dacron patch from the aortic side. At follow-up, he was asymptomatic with no left-to-right shunt.

Anatomic and functional aortic valvuloplasty for correction of aortic valve prolapse in ventricular septal defect with aortic insufficiency.

We describe a new procedure of aortic valvuloplasty for aortic valve prolapse in ventricular septal defect with aortic insufficiency syndrome. This technique allows an anatomic and functional aortic valve reconstruction that prevents late failure of aortic valve repair and reoperation. Midterm results demonstrate the feasibility and durability of this new procedure.

Aortic regurgitation caused by rupture of the abnormal fibrous band between the aortic valve and aortic wall.

This report documents the sudden onset of aortic regurgitation (AR) by an exceptional cause. A 68-year-old woman suddenly experienced general fatigue, and AR was diagnosed. One year later, we performed aortic valve replacement. At surgery, three aortic cusps with a larger noncoronary cusp had prolapsed along with a free-floating fibrous band that had previously anchored the cusp to the aortic wall. Its rupture had induced the sudden onset of AR. There was no sign of infectious endocarditis. We performed successful aortic valve replacement.

[Concealed infective endocarditis].

The occurrence of concealed infection or "natural remission" in infective endocarditis was investigated microscopically in 35 consecutive patients (36 valves) with infective endocarditis (between April 1987 and May 1995). Four patients were considered as having concealed or silent infective endocarditis. Preoperative diagnosis of these patients was mitral valve prolapse, rheumatic aortic valve stenosis with insufficiency, aortic valve prolapse and hypertrophic cardiomyopathy, respectively. These patients did not present with any clinical signs of infective endocarditis such as cardiac murmur and ventricular dysfunction. Histological examination of the excised valves revealed valvular perforation, small round cell infiltration, neovascularization, remnants of vascular smooth muscle cells, and organizing vegetations. These findings are consistent with the histological findings of infective endocarditis. Latent infective endocarditis may be present without inflammatory manifestation.

Severity indices of right coronary cusp prolapse and aortic regurgitation complicating ventricular septal defect in the outlet septum: which defect should be closed?

BACKGROUND: The factors that may determine the evolution of right coronary cusp prolapse (RCCP) and regurgitation (AR) associated with a ventricular septal defect in the outlet septum (outlet VSD) have not been clarified. METHODS AND RESULTS: The Doppler echocardiograms of 316 patients were grouped according to both the development of RCCP, and the values of the right coronary cusp deformity index (RCCD) and the right coronary cusp imbalance index (R/L). All detected AR was /=0.30 or R/L >/=0.30. Rupture of the sinus of Valsalva was identified in patients with RCCD >/=0.30. A significantly large number of patients with both RCCD >/=0.30 and R/L >/=1.30 (p<0.01), and a few patients with either RCCD >/=0.30 or R/L >/=0.30 underwent aortic valvuloplasty or replacement. Operative outcome for AR /=0.30 or R/L >/=1.30.

Imbalance of cusp width and aortic regurgitation associated with aortic cusp prolapse in ventricular septal defect.

The Doppler echocardiograms of the aortic valve and associated aortic regurgitation (AR) were reviewed in 72 patients with a ventricular septal defect (VSD). Group I comprised 13 patients without any deformity of the aortic cusp for > or = 10 years, group 2 included 35 patients who did not develop AR for > or = 10 years after right coronary cusp prolapse (RCCP) was first detected, group 3 comprised 11 patients with RCCP and AR in whom the AR remained subclinical for > or = 10 years, and group 4 was 13 patients who underwent surgical treatment because of moderate to severe AR. The cusp imbalance index [width of right (R) or non- (N) coronary cusp/width of left coronary cusp (L)] was compared among the 4 groups. R/L or N/L was larger in group 4 than in groups 1-3; R/L exceeded 1.30 in all the patients in group 4, whereas it was less than 1.30 in all the atients in groups 1-3. Two patients in group 4 with non-coronary cusp prolapse had an N/L greater than 1.50. No other patients in any group had an N/L larger than 1.20. An imbalance of cusp width may predict possible progressive deterioration of AR.

[Valvular prolapse in autosomal dominant polycystic kidney]. / Il prolasso valvolare nel rene policistico autosomico.

BACKGROUND: The involvement of the cardiovascular system in hereditary connective tissue disorders has been known for a long time and produces abnormalities that usually affect valves and arterial walls. Valvular diseases in typical autosomal dominant illnesses such as polycystic kidney (PK) have recently been reported. METHODS: To test the prevalence of valvular prolapses in this disease, we studied [with clinical, chest x-ray, electrocardiographic, and echocardiographic (monobidimensional and Doppler) examination] 21 subjects with PK (Group A) and 34 unaffected relatives (Group B). 36 subjects comparable in sex, age, blood pressure, body build and renal function, with other kinds of kidney diseases, were the control group (Group C). RESULTS: In group A, 7 subjects (33.3%) were found affected by mitral valve prolapse (MVP), of whom 3 also had mitral incompetence, 4 tricuspid valve prolapse and 2 aortic cusp prolapse with mild valvular insufficiency. Another 2 patients had evidence of mitral and aortic incompetence, respectively, without valvular prolapse, annulus ectasia or morphological features of a rheumatic valvular disease. In Group B, 8 subjects (23.5%) had MVP; In Group C, MVP was seen in only 1 patient (2.7%) and aortic incompetence in one other. Statistical analysis confirmed the significance of the greater prevalence of MVP in group A and B in comparison with group C (p < 0.01). CONCLUSIONS: The high prevalence of valvular abnormalities in autosomal dominant PK suggests a common genetic disorder producing a defect in the extracellular matrix. An alternative hypothesis is that of two discrete yet adjacent genes.

Anomalous right coronary artery from the main pulmonary artery in a patient with double-chambered right ventricle.

We describe a rare case of double-chambered right ventricle (DCRV) in a 32-year-old female presenting to the echocardiography lab for evaluation of congenital heart disease. We identified a unique constellation of findings, including the DCRV, a perimembranous ventricular septal defect, aortic valve prolapse, patent foramen ovale, and an anomalous right coronary artery coming off the main pulmonary artery. To the best of our knowledge, this is the first reported case describing the association of an anomalous right coronary artery coming off the main pulmonary artery in a patient with DCRV.