Tricuspid Valve Prolapse Research Articles

Impact of Age and Gender on Cardiac Pathology in Children and Adolescents With Marfan Syndrome

Cardiac pathologies are the major aspect in the treatment strategies for Marfan syndrome (MFS). In this progressive disease, less is known about manifestations and progression of cardiovascular symptoms in children. To define a certain decision regarding therapeutic options, knowledge concerning the onset of cardiovascular findings is essential. From 1998 to 2011, suspected pediatric Marfan patients were subjected to a standardized diagnostic program. Cardiovascular findings were analyzed in terms of age at first clinical manifestation, prevalence and gender differences, morbidity, mortality, and treatment. Marfan syndrome was diagnosed in 82 patients (46 boys; mean age at diagnosis, 9.0 ± 5.7 years). At first presentation, aortic root dilation was found in 56 % of patients, mitral valve prolapse in 31 %, whereas pulmonary artery dilation was detected in 22 % and tricuspid valve prolapse in only 17 % of patients. Aortic (2.5 %) and mitral valve regurgitations (22 %) are significantly correlated with aortic root dilation (p < 0.01) and mitral valve prolapse (p < 0.05) but without relevant progression during childhood. Prophylactic medication was initiated for 42 % of the patients (mean age, 8.0 ± 4.5 years) because of progressive aortic root dilation. Aortic dissection did not appear. Aortic root surgery was needed for 4 % of the patients. Gender-specific differences in cardiovascular findings, progression of disease, or treatment did not appear. Comparable with adults, aortic root dilation is the most frequent cardiovascular finding in children and associated with relevant morbidity, whereas aortic and mitral valve regurgitation are of minor clinical relevance. Manifestation at an early age and slow progression of cardiovascular findings underscore the necessity of repeated echocardiographic examinations for early diagnosis and start of prophylactic treatment.

Cardiovascular findings of children with Marfan syndrome

Objectives: The aim of our study is to investigate the frequency of structural heart diseases in patients with Marfan syndrome (MS) and to reveal the importance of clinical follow-up in MS. Materials and methods: Study population consisted of 17 patients admitted to the Pediatric Cardiology department between January 2005 and March 2010 with the diagnosis of MS according to the Ghent criteria. Patients were evaluated for the eye, genetic and the cardiovascular system abnormalities. Physical examination findings, echocardiographic, and radiological examinations of the patients were evaluated retrospectively. Results: Of the 17 cases, 9 were girls and 8 were males, ages ranged from 1 month to 17 years (mean 9.7 years). There was a second degree of kinship between mothers and fathers in 5 patients. Respiratory distress, syncope, chest pain and palpitation were the most seen in the presentation complaint of the patients. Skeletal findings observed in 13 patients, 4 patients had subluxation of the lens. Two patients had positive family history. When cardiovascular findings were examined, there were aortic root dilatation with mitral valve prolapse and/or tricuspid valve prolapse in 8 patients, mitral valve prolapse in 3 patients only aortic root dilatation in 3 patients and mitral valve prolapse and tricuspid valve prolapse in 3 patients. Mean follow-up period was 2.6 years, aneurysm and rupture of the aorta wasn’t observed during this period. Conclusion: In patients with Marfan syndrome, regular follow-up and cardiological evaluation should be done because significant structural heart diseases can be seen in these patients.

Pectus excavatum: echocardiography and cardiac MRI reveal frequent pericardial effusion and right-sided heart anomalies

In patients with pectus excavatum (PEX), echocardiographic assessment can be difficult. There are little data on the impact of the chest deformity on echocardiographic findings and comparison of data obtained by echocardiography (echo) with cardiac magnetic resonance imaging (CMR) in PEX. In a prospective study, cardiac anomalies in PEX were analysed by echo and compared with CMR in consecutive patients with PEX referred for echo. If they agreed to participate, the patients were referred for CMR and included if the pectus index was ≥3.0 by CMR. Also, clinical data and electrocardiogram tracings were analysed. There were 18 patients (13 females; 72%), with a mean age of 53±16 years; mean pectus index was 4.7 (range: 3-7.3). Echo showed haemodynamically insignificant pericardial effusion in six patients (33%), tricuspid valve prolapse in five (28%), right ventricular (RV) localized wall motion anomalies (WMA) in five (28%) and diminished RV systolic function in two (11%); no patient had RV dilatation. CMR demonstrated cardiac displacement to the left in 9 patients (50%); minimal pericardial effusion was seen in 10 patients (56%; P value=0.13 compared with echo), RV localized WMA in 6 (44%; P value=1.0), diminished RV systolic function in 8 (44%; P=0.07), and RV dilatation in 5 (28%; P=0.06). A completely normal cardiac examination was found in six patients by echo (33%) and in 2 (11%) using CMR. Although some signs of arrhythmogenic RV cardiomyopathy (ARVC) were present, no patient fulfilled the ARVC criteria. In severe PEX, haemodynamically insignificant pericardial effusion, tricuspid valve prolapse and other RV anomalies possibly due to RV displacement are frequent as demonstrated by both CMR and echo. The cardiac assessment by echo and CMR did show discrepancies; however, they were not significant.

Isolated tricuspid valve prolapse : identification using two- and three-dimensional echocardiography and transoesophageal echocardiography

We present a case of isolated prolapse of the tricuspid anterior leaflet in an asymptomatic 34-year-old man who was referred to our hospital for a routine check up. We performed two-and three-dimensional transoesophageal echocardiography (TEE). We found three-dimensional TEE a useful, non-invasive tool that can provide additional information to two-dimensional echocardiography in the assessment of tricuspid valve prolapse.

Tricuspid Valve Repair with Artificial Chordae in a 72-Year-Old Woman

Expanded polytetrafluoroethylene sutures have been used for the replacement of chordae tendineae since 1985, especially for mitral valve prolapse. There are only a few reports of artificial chordae tendineae to treat tricuspid valve regurgitation. We report on a 72-year-old woman in NYHA class III preoperatively, who underwent successful tricuspid valve repair after preoperative echocardiography revealed tricuspid valve regurgitation grade IV, caused by prolapse of the anterior leaflet (A1-A2) and annular dilatation. Tricuspid valve repair was performed using artificial chords consisting of two polytetrafluoroethylene sutures and a ring annuloplasty. Postoperative echocardiography revealed mild tricuspid valve regurgitation of less than 1°, even after three years. Gore-Tex® sutures as used in mitral valve repair are an excellent option to replace chordae tendineae in tricuspid valve prolapse. This approach represents a safe and effective technique for tricuspid valve repair.

Echocardiographic findings in children with Marfan syndrome

The typical cardiac manifestations of Marfan syndrome are aortic regurgitation with progressive dilatation of the aortic root, which may cause dissection and rupture of the ascending aorta, mitral valve prolapse and mitral valve regurgitation. In this study, we aimed to show echocardiographic findings in 11 patients with Marfan syndrome. Diagnosis of Marfan syndrome was based on the Ghent criteria. All patients had a full echocardiographic evaluation. During the evaluation, we investigated the presence of mitral valve prolapse, mitral valve regurgitation, tricuspid valve prolapse, dilatation of the aortic root, and aortic regurgitation. Eleven patients were diagnosed as Marfan syndrome (seven male, four female, age 4-14 years). All had mitral valve prolapse (nine with mitral valve regurgitation). Among these 11 patients, seven had accompanying tricuspid valve prolapse, six had dilatation of the aortic root and two had aortic regurgitation. Eleven patients in our clinic were diagnosed as Marfan syndrome since they had distinct characteristics of marfanoid phenotype. Echocardiographic evaluation of these patients showed marked heart valve involvement. In Marfan syndrome, it is known that the aortic valve is affected following mitral valve involvement. In our experience, aortic root dilatation is less common. However, particular attention should be given to following up aortic root status with noninvasive echocardiography to institute measures to prevent complications.

Frequency and Age-Related Course of Mitral Valve Dysfunction in the Marfan Syndrome

Mitral valve (MV) prolapse (MVP) has a high prevalence of 2% to 3% in the general population and thus constitutes the most common cause of severe nonischemic MV regurgitation (MVR). MVP is also common in persons with the Marfan syndrome. However, to date, a large-scale population-based cohort study using modern echocardiographic techniques has not been performed, and the frequency of MVP and the relation of MV dysfunction and age have not been investigated. Therefore, we conducted a population-based cohort study of 204 patients (108 males and 96 females, aged 31.2 ± 16.4 years) with classic Marfan syndrome. We performed echocardiographic follow-up of 174 patients for a mean of 4.4 ± 4.3 years. On the initial or subsequent echocardiographic scan, MVP was present in 82 patients (40%), severe MVR in 25 (12%), and MV endocarditis in 5 patients (2.5%). At 30 years of age, the Weibull cumulative distribution was 42.6% (95% confidence interval [CI] 36% to 50%) for MVP, 56.5% (95% CI 49.3% to 64%) for MVR of any degree, 6.7% (95% CI 3.9% to 11.3%) for severe MVR, and 0.92% (95% CI 0.21% to 3.91%) for MV endocarditis. The cumulative hazard for severe MVR and MV endocarditis was estimated to increase with age. MVP was associated with dural ectasia (p = 0.01), ectopia lentis (p = 0.02), and skeletal involvement (p <0.001). Severe MVR was related to tricuspid valve prolapse (p = 0.002) and to the sporadic form of the Marfan syndrome (p = 0.006). In conclusion, MVP was comparatively frequent in patients with the Marfan syndrome and carries an increased risk of progression to severe MVR and endocarditis, especially in older adults.

ACUTE EXACERBATION OF RIGHT HEART FAILURE BY TRICUSPID VALVE PROLAPSE IN PULMONARY ARTERIAL HYPERTENSION

PURPOSE: Tricuspid regurgitation (TR) in pulmonary arterial hypertension (PAH) is a well recognized corollary of right ventricle (RV) failure. Right ventricular systolic pressure (RVSP) greater than 55mm Hg usually results in functional TR. Isolated tricuspid valve prolapse (TVP) is a rarely described clinical entity. We describe three PAH subjects who developed acute decompensated right heart failure due to TVP with a poor clinical outcome in two.

A Heart-shaped Mass Inside a Heart: Echocardiographic Diagnosis, Pathology, and Surgical Repair of a Flail Tricuspid Valve Caused by a Large Blood-filled Cyst

A 55-year-old white man with a history of a systolic murmur since childhood was found to have significant tricuspid regurgitation 15 years ago. Two years before presentation, an echocardiogram revealed an ill-defined, large cystic mass on his tricuspid valve, tricuspid valve prolapse, and severe tricuspid regurgitation with normal right-sided function. No treatment was recommended because of the asymptomatic status of the patient. The patient was referred to our institution with 3 weeks of exertional dyspnea, orthopnea, and paroxysmal nocturnal dyspnea. An outside transthoracic echocardiogram showed severe tricuspid regurgitation, tricuspid valve prolapse, and a cystic mass associated with it. The right ventricular systolic pressure was normal. On physical examination, his vital signs were normal with the presence of jugular venous distention and prominent flutter waves in his internal jugular profile. His lungs were clear. He had a nondisplaced, nonsustained, apical impulse with normal S1 and S2 and no S3 or S4. There was a grade 3/6 holosystolic murmur at the left lower sternal border with a positive Carvallo’s sign. He had no lower extremity edema. An electrocardiogram showed atrial flutter with variable conduction. A transesophageal echocardiogram revealed normal left ventricular size and function with moderate right ventricular dilation and borderline decrease in right ventricular systolic function. There was a large, echolucent mass composed of several lobes on the atrial surface of the anterior tricuspid leaflet that measured 25 by 30 mm and prolapsed into the right atrium during systole and the right ventricle during diastole (Video 1 and Figure 1). The mass was cystic and the shape resemb heart when contrast (agitated saline) was administered (Video 2 and Figure 2). An eccentric posterior medi of severe tricuspid valve regurgitation (Video 3) was associated to the mass with severe prolapse and ruptured

Flail mitral and tricuspid valves due to myxomatous disease

Myxomatous disease generally affects mitral valve. However, tricuspid valves also can be involved in 20% of the myxomatous mitral valve disease. Valve prolapse, elongation of chordae and chordae rupture are generally seen complications of the myxomatous disease. There are some reports about severe tricuspid regurgitation due to tricuspid valve prolapse and elongated chordae, but no tricuspid and mitral chordae ruptures in the same patient due to myxomatous disease have been reported. In this case tricuspid chordae rupture accompanied to mitral chordae rupture is discussed.

Echocardiographic and Clinical Abnormalities in Patients with Psoriasis

We investigated the incidence and severity of echocardiographic and clinical abnormalities in patients with psoriasis and their relationship to the severity, duration and type of psoriasis and other related factors. A total of 216 psoriasis patients and 216 control subjects were included in this study. Left and right heart dimensions, wall thicknesses, wall motion abnormalities, valvular disturbances, and systolic and diastolic functions were examined using two-dimensional and Doppler echocardiographic techniques. Left ventricular hypertrophy, left ventricular diastolic dysfunction, left ventricular wall motion abnormalities and valvular pathologies, especially mitral and tricuspid valve prolapse, were significantly more frequent in patients with psoriasis. Systolic and diastolic blood pressures were significantly higher in psoriasis patients. Significant correlations were found between: (i) psoriasis vulgaris and tricuspid valve prolapse; (ii) palmo-plantar psoriasis and valvular pathologies; and (iii) disease duration and left ventricular diastolic dysfunction and systolic and diastolic blood pressures. Physicians should be aware that cardiovascular abnormalities are common in patients with psoriasis.

Ebstein's anomaly - review of a multifaceted congenital cardiac condition.

Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects. Since its description in 1866, dramatic advances in diagnosis and therapy have been made. In this review, we describe current diagnostic criteria and classification, natural history, clinical features, and prognosis, typical echocardiographic features and pathologic findings, and the spectrum of associated cardiac malformations including left heart anomalies associated with EA. Differences between Ebstein-like changes associated with congenitally corrected transposition and EA are described. The spectrum of typical ECG and conduction system changes, arrhythmias including accessory pathways and ectopic atrial tachycardias related to EA are also reviewed. Differential diagnosis of EA is discussed including tricuspid valve dysplasia and prolapse as well as arrhythmogenic right ventricular cardiomyopathy. The review describes management options in EA including catheter interventions, indication for operation and surgical options including tricuspid valve repair and replacement. Overall, EA is a complex congenital anomaly with a broad pathologic-anatomical and clinical spectrum and no two patients are alike. Therefore, precise knowledge of the different anatomic and hemodynamic variables, associated malformations and management options are essential. Management of EA patients is complex. Thus it is important that these patients are regularly seen by a cardiologist with expertise in congenital heart disease.

Ebstein's anomaly - review of a multifaceted congenital cardiac condition.

Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects. Since its description in 1866, dramatic advances in diagnosis and therapy have been made. In this review, we describe current diagnostic criteria and classification, natural history, clinical features, and prognosis, typical echocardiographic features and pathologic findings, and the spectrum of associated cardiac malformations including left heart anomalies associated with EA. Differences between Ebstein-like changes associated with congenitally corrected transposition and EA are described. The spectrum of typical ECG and conduction system changes, arrhythmias including accessory pathways and ectopic atrial tachycardias related to EA are also reviewed. Differential diagnosis of EA is discussed including tricuspid valve dysplasia and prolapse as well as arrhythmogenic right ventricular cardiomyopathy. The review describes management options in EA including catheter interventions, indication for operation and surgical options including tricuspid valve repair and replacement. Overall, EA is a complex congenital anomaly with a broad pathologic-anatomical and clinical spectrum and no two patients are alike. Therefore, precise knowledge of the different anatomic and hemodynamic variables, associated malformations and management options are essential. Management of EA patients is complex. Thus it is important that these patients are regularly seen by a cardiologist with expertise in congenital heart disease.

Acromegalia asociada a prolapso mitral y tricuspídeo. Descripción de un caso

A 65 year-old male with severe systolic mitral and tricuspid valve prolapse, associated with long-standing acromegaly is reported. The non published association could be caused by the effect of growth hormone on the valve connective tissue.

Relation between QT dispersion and ventricular arrhythmias in uncomplicated isolated mitral valve prolapse.

Complications of mitral valve prolapse (MVP), among which serious ventricular arrhythmia and sudden death are of major importance, affect many individuals due to the high incidence of MVP itself in the community despite the actual low incidence of these complications. The present study investigated the incidence and distribution of ventricular arrhythmias according to their severity and relationship with the QT interval and dispersion of repolarization in uncomplicated isolated MVP (IMVP) cases. Fifty-eight uncomplicated IMVP patients, 33 patients with accompanying tricuspid valve prolapse (TVP), to compare its relationship with ventricular arrhythmia, and 60 age- and sex-matched control subjects were enrolled in the study. Individuals with accompanying cardiac or systemic disease, or who were on drug therapy that could potentially affect QT characteristics, were excluded. The incidence of ventricular arrhythmia was 48% in the IMVP group and 64% in the TVP group; the difference was statistically insignificant. In addition, the differences of the QT and Q peak T values were insignificant, whereas QT dispersion (QTd) and Q peak T dispersion (QpeakTd) values were significantly higher in the patient group (60+/-14, 54+/-14 ms, respectively) compared with the control group (42+/-10, 38+/-10 ms, respectively, p<0.001). Complex ventricular arrhythmias (Lown Grade > or =III) in the IMVP group had a significant relationship with QTd and QpeakTd (p<0.001), but not with QT or QpeakT. As a result of the study, it is concluded that TVP accompanying MVP does not increase the incidence of ventricular arrhythmia, that ventricular arrhythmia is related to QT dispersion rather than QT interval in IMVP, that the QT dispersion is a fairly good marker for identifying the high-risk group for serious ventricular arrhythmia and sudden death, and that QpeakT dispersion measurement is an additional indicator that could be an alternative when QT is difficult to determine in conditions such as high heart rate or the presence of U wave.

Mitral and Tricuspid Valve Prolapse: A Common association?

Mitral and Tricuspid Valve Prolapse: A Common association?

Mitral and tricuspid valve prolapse: a common association?

Mitral and tricuspid valve prolapse: a common association?

Mimics of Ebstein’s anomaly

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein’s anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein’s anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein’s anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein’s anomaly but were found to have other abnormalities of the tricuspid valve or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein’s anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein’s anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein’s anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein’s anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (≥8 mm/m 2 ) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein’s anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (≥8 mm/m 2 ) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein’s anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein’s anomaly had important surgical implications. (Am Heart J 1997;134:508-13.)

Echocardiographic Changes in Cases of Intermittent Acute Porphyria.

Intermittent acute porphyria (IAP) is an inborn metabolic disorder of biosynthesis of haeme, characterized by increased excretion of porphyrin or porphyrin precursor in urine and clinically by gastrointestinal, neuro-psychiatric and cardiovascular manifestations. Significant observations were made on echocardiographic examination of 25 IAP patients in our study. Significant decrease in ejection fraction (48.4 +/- 7.9, control group 63.0 +/- 7.5, P < 0.001) and percentage of fractional shortening (23.5% +/- 6.87%, control group = 36.4 +/- 4.26, P </=.001) was observed in IAP, this shows derangement in left ventricular systolic functions in IAP. The study also shows significant thickening of IVS (1.22 +/- 0.20, control group 0.88 +/- 0.12 cm, P </= 0.001). The most interesting finding was the significantly increased incidence of mitral valve and/or tricuspid valve prolapse in 15 (60%) patients of IAP. Thickening of AML, PML, and calcification of AML were also observed.

Abnormal ambulatory electrocardiographic findings in patients with the Marfan syndrome.

The aim of this study was to assess the prevalence of cardiac dysrhythmias and abnormalities of conduction and repolarization in the Marfan-syndrome (MFS). Forty-five adult MFS patients (25 men) and healthy age and sex matched controls. A 24-h ambulatory electrocardiogram was recorded. There was no difference in heart rates between the two groups. Two MFS patients had atrial fibrillation. The median number of premature atrial beats was 12/24 h in the MFS group vs. 6/24 h in the controls (P < 0.05), and the respective medians of premature ventricular beats were 17/24 h vs. 1/24 h (P < 0.001). Five patients but no healthy person had salvos of > or = 3 premature ventricular complexes (P < 0.05). Ventricular premature beats with R on T configuration were recorded in nine patients but in none of the control subjects (P < 0.05). Both PQ and QT intervals at heart rates of 60, 80 and 100 beats min-1 were longer in the MFS group compared with healthy persons (P < 0.005). Also ST segment depression was seen more often in the MFS group (17/43 vs. 6/45; P < 0.05). In patients with MFS, the findings at ambulatory electrocardiography showed no association with echocardiographically determined aortic root diameter, left atrial diameter or left ventricular diameters, wall thickness and systolic function. Nor did the electrocardiographic findings correlate with the presence of mitral or tricuspid valve prolapse. Patients with MFS have a higher prevalence of cardiac dysrhythmias than healthy persons. Likewise they have prolonged atrio-ventricular conduction time and disturbed depolarization as suggested by longer QT intervals and more common ST segment depression.