Congenital mitral regurgitation with Down syndrome.

A 19-year-old man with Down syndrome had congenital mitral regurgitation. He had slight mental retardation and difficulty in performing independent oral drug administration. We planned mitral valve plasty to avoid postoperative anticoagulant therapy. The mitral valve lesions were complicated. A prolapsed anterior mitral leaflet, shortened posterior leaflet, abnormal cleft, abnormal papillary muscle, and enlarged annulus were observed. Successful mitral valve plasty was performed using several techniques. The postoperative course was uneventful, and no significant mitral regurgitation was found.

Echocardiography of congenital mitral valve disorders: echocardiographic-morphological comparisons.

I surveyed our echocardiographic database of the years between 1998 and 2012 for congenital abnormalities of the mitral valve in patients over 14 years. A total of 249 patients with mitral valve abnormalities were identified. Abnormalities included clefts in the mitral valve in 58 patients, double orifice of the mitral valve in 19, mitral stenosis with two papillary muscles in 72, and mitral stenosis with one papillary muscle in 51 patients. Supravalvar rings were found in 35 patients with a single papillary muscle, and mitral stenoses with two papillary muscles were found in 22 patients. Mitral prolapse occurred in 44 patients and mitral valvar straddle in five patients. The patients were evaluated by all modalities of ultrasound available over the course of time. Although some lesions were isolated, there were many lesions in which more than one mitral deformity presented in the same patient. The patients are presented showing anatomical correlation with autopsy specimens, some of which came from the patients in this series, and others matched to show correlative anatomy. These lesions remain rare as a group and continue to have high morbidity and mortality.

[Fifteen years experience of the use of magnesium preparations in patients with mitral valve prolapse].

We followed for 15 years 31 patients with mitral valve prolapse (MVP) who during follow-up regularly took orotic acid (1500 mg/day) for 3 months twice a year. We revealed peculiarities of dynamics of clinical picture, their interrelation with phenotypic manifestations of connective tissue dysplasia, changes of electrocardiogram, structure of valvular apparatus of the heart, state of vegetative homeostasis, changes of levels and 24-hour profile of arterial pressure, tone of sympathetic and parasympathetic parts of vegetative nervous system. We noted significant reduction of mean and maximal heart rate, number of episodes of tachycardia, duration of QTc intervals, incidence of paroxysmal supraventricular and ventricular extrasystoles. We fixed statistically significant lowering of maximal systolic and diastolic arterial pressure, hypertensive burden and elevated variability of systolic and diastolic arterial pressure. Data of retrospective analysis showed absolute normalization of these parameters in all studied patients. Decrease of the tone of sympathetic part of vegetative nervous system was also established. There was 2 to fold decrease of number of persons with sympathicotonia, 3 to fold increase of those with vagotonia, and 5 times increase of number of patients with equal tone of sympathetic and parasympathetic parts. Regular use of magnesium salt of orotic acid was associated with significant elevation of quality of life of patients with MVP. Clinically valuable improvement of work and social life scores was noted in 54.8%, of personal life score - in 45.2% of individuals. In half of patients with MVP index of efficacy of therapy with orotic acid was significant.

The prospective follow-up of the natural course of interatrial communications diagnosed in 847 newborns.

AIMS: The aim of this study was to evaluate the prevalance of interatrial communications (IACs) and IAC types in a large series of newborns, to establish the incidence of spontaneous closure of IACs, to determine the relationship between spontaneous closure and the size and type of IACs, and to investigate the incidence of mitral valve prolapse (MVP) and atrial arrhythmia in newborn infants with atrial septal aneurysm (ASA). METHODS AND RESULTS: Between 2000 and 2001, a total of 1100 asymptomatic and term newborns were evaluated. Those who had congenital heart diseases and failed to attend the follow-up visits were excluded from the study. The remaining 847 newborns were followed until the closure time or in those where closure did not occur, for a maximum time of 45 months (mean 25 +/- 3 months, range 1-45 months). The mean age at diagnosis was 1.7 +/- 1.4 days (range 1-7 days). According to echocardiographic evaluation, cases were classified into four groups based on the initial size of IAC and into three groups based on the type of IAC. At the end of the 45th month IACs were closed spontaneously in 98.6% of the cases. There was significant relationship between the diameter of IAC and the timing of the closure (P < 0.01). The closure time in the cases with ASA was significantly longer than the cases with valve-like opening and multiple fenestration (P < 0.01). In female newborns, the defects remained open for a significantly longer period than male newborns (P = 0.0397). There was no significant relationship between ASA and atrial arrhythmias (P = 0.294). None of the newborns had MVP. CONCLUSION: The cases with IACs < 3 mm do not need follow-up. However, the cases with IACs > 3 mm do need to be followed until the defect closes completely. Those with ASA should be followed-up regularly, because these defects can remain open. Spontaneous closure occurs significantly earlier in cases with valve-like opening and multiple fenestration.

Congenital mitral valve surgery: techniques and results.

PURPOSE OF REVIEW: Congenital lesions of the mitral valve are rare. Conservative surgery is recognized as the best option. In complex anatomy, however, replacement is the only solution to achieve an acceptable result. This review aims to study the long-term follow-up of classical treatments, conservative or replacement, and to examine new technical advances. RECENT FINDINGS: The long-term results of conservative surgery are confirmed with a low incidence of reoperation except in mitral valve stenosis. The Ross II operation using a pulmonary autograft is a difficult technique that may be useful in the youngest patient group when prosthetic devices cannot be used. SUMMARY: In the last few years, surgery of congenital mitral valve lesions has gained from echocardiography, which shows the exact function and anatomy of the mitral valve. The tendency is to avoid multistage operations. Valve replacement by biologic material (Ross II) is still under clinical evaluation.

Is mitral valve prolapse a congenital or acquired disease?

The prevalence of mitral valve prolapse (MVP) at birth was studied in 1,734 consecutive newborns without congenital structural heart disease. We have not identified any case of an unequivocal pattern of MVP using auscultatory and echocardiographic diagnostic criteria. Our data argue for the concept that MVP is an acquired disease.

Double orifice in prolapsing mitral valve.

A prolapsing mitral valve with a double orifice ('hole type') was documented by echocardiography in a 35-year-old male. His symptoms were associated to supraventricular ectopic beats and persisted unchanged during a 3-year follow-up. This malformation is usually considered benign but, as fragmentation of the atrioventricular conduction tissue was reported in some cases, a periodic observation is advisable.

[Surgical treatment of mitral prolapse with chordae plasty].

Surgical treatment of 8 cases of anterior mitral leaflet prolapse was reported. Of the 8 cases, chordae elongation was occurred in 2, chordae rupture in 6, and congenital in 6, rheumatic in 2. Chordae reimplanting was performed in 5 cases, chordae shortening in 2, chordae prosthesis with surgical suture in 1, and combined annuloplasty in 7 of the 8 cases. The result of mitral plasty was better in congenital mitral prolapse. Indications of this technique and postoperative management were listed.

Currarino-Silverman syndrome (pectus carinatum type 2 deformity) and mitral valve disease.

Currarino-Silverman syndrome is a rare disorder characterized by premature fusion of manubrio-sternal joint and the sternal segments, resulting in a high carinate chest deformity; it is frequently associated with congenital heart disease. Among the various heart lesions reported in this syndrome, mitral valve disease and coarctation of the aorta have not yet been described (to our knowledge). Our report consists of five children with this syndrome, four of whom had mitral valve disease, with an associated coarctation of the aorta in one patient. The fifth patient had an innocent heart murmur.

[Transient myocardial ischemia and isolated congenital mitral valve prolapse in an infant]. / Ischémie myocardique transitoire et prolapsus valvulaire mitral congénital isolé chez un nourrisson.

The investigation of a cardiac murmur in a 9 month old infant led to the clinical, echo and angiographic diagnosis of congenital mitral regurgitation due to isolated mitral valve prolapse, whereas the electrocardiogram showed signs of inferior myocardial ischaemia which regressed in a week. The valvular dysfunction of mitral valve prolapse has been held responsible for coronary spasm ischaemia and infarction in adults. This complication has not been previously reported to our knowledge in infancy.

Coronary aneurysms in a case of Ehlers-Danlos syndrome.

An asymptomatic 48 year old man had transient T-wave inversion in the anterolateral leads after nonpenetrating chest trauma. Both the patient and his 6 year old son showed the typical skin hyperelasticity and joint hyperextensibility of the Ehlers-Danlos syndrome, suggesting autosomal dominant inheritance. The coexisting cardiac abnormalities of the patient included multiple coronary aneurysms, mitral valve prolapse with slight regurgitation, fusiform dilatation of the ascending aorta with moderate insufficiency and left anterior hemiblock. A routine noninvasive cardiovascular evaluation should be performed in all patients with Ehlers-Danlos syndrome in order to exclude valvular heart disease and dilatation of the aortic root or of other vessels, including the coronary arteries.

An infant with Marfanoid phenotype and congenital contractures associated with ocular and cardiovascular anomalies, cerebral white matter hypoplasia and spinal axonopathy.

An infant is presented with a Marfanoid phenotype and congenital contractures. In addition to this she showed severe neurological and ocular abnormalities. Cardiac insufficiency due to mitral and tricuspidal valve prolapse caused her death at the age of 6 months. Postmortem examination showed axonal pathology of the anterior horns and roots of the spinal cord, and white matter hypoplasia of the brain.