Open-heart valvular surgery in children. Apropos of 127 cases

In view of the frequency and the severity of rheumatic heart disease (RHD) and of endomyocardial fibrosis (EMF) in the african child, the indication of a valvular surgical procedure is frequent in Abidjan. Between 1978 and 1983, 127 open-heart procedures have been performed in 113 patients. The ages ranged from 2 to 15 years, with a mean of 11 years. There were 67 RHD, 37 EMF, 9 bacterial endocarditis. The status of the patients was severe, there was a cardiomegaly (mean CTR of 0.70). 3 patients were in atrial fibrillation. The patients underwent 97 valve replacements (70 mitral, 18 tricuspid, 9 aortic) essentially by a bioprosthesis (n : 87). A mitral valvuloplasty was done in 27 cases. Associate procedures have been employed including : 35 endocardectomies, 11 aortic valvuloplasties, 6 tricuspid annuloplasties. 12 patients have been reoperated for bacterial endocarditis of a mitral prosthesis (n : 4), calcification of a mitral bioprosthesis (n : 5), failure of mitral valvuloplasty (n : 3). There were 13 deaths (10.2 p. 100 mostly in EMF surgery : 7 deaths (5.5 p. 100). No patient with a bioprosthesis received anticoagulants post-operatively. All surviving patients have been improved, with a mean post-operative follow-up of 32 months. There were 11 late deaths (8.6 p. 100). The authors discuss several points including : severity of children valvular diseases in tropical areas; necessity to use the bioprosthesis in this area of the world; increasing tendency to conservative surgery in the mitral and also the aortic position.     

Reconstructive mitral valvuloplasty. Results apropos of 53 cases

In the last three years, 53 patients have undergone a valvuloplasty according to the Carpentier's principles. The patients age ranged from 5 to 35 years, with a mean of 14.3 years. The aetiology was congenital in one case, RHD in 45 cases, and endomyocardial fibrosis (EMF) in 7. The pre-operative condition was severe with 25 patients in class III and 10 patients in class IV. There was a cardiomegaly with a mean CTR of 0.70 (extremes 0.50 and 0.85). The mitral lesions were pure mitral insufficiency in 49 cases, and mixed lesions with predominant insufficiency in 4 cases. There were also 6 aortic and 4 tricuspid significant insufficiencies. The patients have had a valvuloplasty with chordae shortening (n: 46) valvular resection (n: 3), chordae resection (n: 8), annuloplasty without ring (n: 38) or with ring (n: 2). There were associated procedures: endocardectomy (n: 7), aortic valvuloplasty (n: 3), aortic valve replacement (n: 3), tricuspid annuloplasty (n: 4). There were 3 post-operative deaths in children in class IV, with cardiomegaly (CTR 0,80), and systemic pulmonary hypertension. There were 3 mitral valve replacements (MVR) in the first month for failure of the plasty, 2 late MVR for endocarditis and rheumatic evolution. There were 3 mitral valve replacements (MVR) in the first month for failure of the plasty, 2 late MVR for endocarditis and rheumatic evolution. There were 2 late deaths (recurrent mitral insufficiency, serum hepatitis), Among 43 long-term follow-up of patients with a plasty (mean follow-up 18 months), the result has been very good 39 times. There were 4 patients with a significant residual mitral incompetence.(ABSTRACT TRUNCATED AT 250 WORDS)     

Percutaneous valvotomy in the combination of mitral and tricuspid valve stenosis. Report of 3 cases

The authors report the results of a series of 3 cases of double valvuloplasty with a balloon catheter in young patients with combined mitral and tricuspid stenosis. Haemodynamic and Doppler echocardiographic evaluation after the procedure showed comparable results to those of surgical commissurotomy without significant secondary valvular regurgitation. Clinical and echocardiographic follow-up showed that valvular opening remained satisfactory in the 2 cases examined. Percutaneous valvuloplasty would seem to be a valuable alternative to surgical commissurotomy in selected patients with combined mitral and tricuspid valve stenosis.     

复发性心脏瓣膜病再次外科治疗效果及其相关因素

目的:回顾性分析对二尖瓣闭式扩张术、瓣膜成形术、瓣周漏、人工机械瓣功能障碍、生物瓣衰坏等原因引起的复发性瓣膜病变进行再次手术的效果和相关因素。方法: 复发性瓣膜病患者331（男143,女188）例,年龄12～73(46±12)岁,两次手术间隔时间2月～25(17±8)年。其中二尖瓣闭式扩张术后再狭窄143例,二尖瓣或主动脉瓣成形术后瓣膜病变复发53例,生物瓣衰坏32例,瓣周漏26例,换瓣术后其它瓣膜病21例,人工瓣膜替换或瓣膜成形术后心内膜炎17例, Ebstein畸形矫治术后三尖瓣关闭不全15例,人工瓣膜机械功能故障9例,室间隔缺损修补术并行瓣膜成形术后心内膜炎7例,完全或部分性心内膜垫缺损和矫正性大动脉转位术后二尖瓣或三尖瓣关闭不全6 例,二尖瓣球囊扩张术2例。再次手术方式为二尖瓣替换术,主动脉瓣替换术,二尖瓣和主动脉瓣替换术,三尖瓣替换术,瓣周漏修补术及三尖瓣成形术等。结果: 全组共死亡27例,占8.2％,早期主要死亡原因为低心排出量综合征、室性心律失常、多脏器功能衰竭、左心室破裂、感染性心内膜炎、肾功能衰竭。随访259例,随访期6月～21（10±7）年,心功能恢复至Ⅰ～Ⅱ级189例。复发性心脏瓣膜病再次手术的危险因素包括术前心功能差、重要脏器功能不全、急诊手术、主动脉阻断时间和体外循环时间长等。结论: 针对再手术相关的危险因素进行积极防治,适时而妥善的外科手术和围手术期处理仍可获良好效果。     

Left atrial electric isolation in the treatment of atrial fibrillation secondary to rheumatic valvular disease]

BACKGROUND. Surgical isolation of the left atrium was performed for the treatment of chronic atrial fibrillation secondary to valvular disease in 100 patients who underwent valve surgery. METHODS. From May 1989 to September 1991, 62 patients underwent mitral valve surgery (Group I), 19 underwent mitral valve surgery and DeVega tricuspid annuloplasty (Group II), 15 underwent mitral and aortic surgery (Group III), and 4 patients underwent mitral and aortic surgery and DeVega tricuspid annuloplasty (Group IV). Left atrial isolation was performed prolonging the usual left paraseptal atriotomy towards the left fibrous trigone anteriorly, and the postero-medial commissure posteriorly. The incision was conducted a few millimeters apart from the mitral valve annulus, and cryolesion were placed at the edges to ensure complete electrophysiological isolation of the left atrium. RESULTS. Operative mortality accounted for 3 cases (3%). In 79 patients (81.4%) sinus rhythm recovered and persisted until discharge from the hospital. No differences were found between the groups (Group I: 80.7%; Group II: 68.5%; Group III 86.7%, Group IV 75% - p = N.S.). Three cases of late mortality (3.1%) were registered. long-term results showed persistence of SR in 71% of Group I, 61.2% of Group II, 85.8% of Group III, and 100% of Group IV. The unique risk factor for late recurrency of atrial fibrillation was found to be a duration of preoperative AF longer than 6 months. CONCLUSIONS. Due to the high success rate in recovering the sinus rhythm, we suggest left atrial isolation in patients with chronic atrial fibrillation undergoing valvular surgery.     

Endomyocardial fibrosis: report of 22 Congolese cases

A study has been made of 22 cases of endomyocardial fibrosis (EMF) observed over a 12-year period. The epidemiological characteristics have been described, and the importance of the echocardiographic findings in the diagnosis of the disease has been emphasized. EMF constitutes 0.3% of the total admissions in the department, 0.9% of the cases of cardiac failure, and 3% of the cases involving subjects under 40 years old. The patient population consisted of 13 men and 9 women with an average age of 35.6 +/- 16.4 years (age range: 8.5-77 years). The diagnosis of EMF was based on clinical, radiological, electrocardiographic, and echocardiographic findings (22 cases), and surgical examination (1 case). In 8 cases, parasitosis (filariasis: the patients came from a known endemic area) with hypereosinophilia was observed. Three of these patients had associated high blood pressure. In 13 subjects, a severe right adiastolic syndrome was noted. Two patients presented with tachycardia, 2 others had signs of overall cardiac failure, and 5 subjects suffered from palpitations connected with arrhythmia. Thoracic X-ray showed cardiomegaly in all cases, lung involvement in 15 cases, normal lungs in 5 cases, and bilateral hilar stasis in 2 cases. Electrocardiography mainly showed endomyocardial fibrosis (15 cases), supraventricular arrhythmia, notably auricular fibrillation (13 cases), and conductive disorders (12 cases), which were frequently associated. Echocardiography showed the presence of EMF in 21 cases (95.5%). Doppler (n = 9 cases) detected tricuspid failure in 9 subjects, and mitral failure in 1 subjects. EMF was exclusively located on the right side in 19 cases, bilateral in 2 cases, and on the left in 1 case, which required surgery. Four patients died (i.e., 1 case of sudden death, 1 case of pulmonary embolism, 1 case of neurological coma, and 1 case of cardiac arrhythmia). The authors, like many others, note the clinical polymorphism of EMF, the predisposition to the disease caused by the presence of helminthiasis, which should be eradicated, the diagnostic value of echocardiography-Doppler, and the efficiency of surgery in the treatment of this condition.     

Pacemaker lead-related tricuspid stenosis successfully treated with percutaneous balloon valvuloplasty guided by 3D echocardiography

The most common etiology of tricuspid stenosis is rheumatic, and in most cases it is associated with valvular regurgitation. Interestingly, there have been reports of tricuspid stenosis without associated valvular regurgitation, mostly related to pacemaker leads. Percutaneous tricuspid valvuloplasty may be a therapeutic alternative to surgery in cases of pure tricuspid stenosis without other concomitant valvulopathies. We report the case of a 52-year-old woman with pacemaker lead-related tricuspid stenosis successfully treated with percutaneous valvuloplasty guided by 3D echocardiography.     

Reoperations in valve surgery. Apropos of 194 cases

Between 1970 and 1985, 194 patients underwent one or several reoperations after conservative valvular surgery (Group A) or valvular replacement surgery (Group B). Group A: comprised 141 patients with a previous history of closed heart mitral commissurotomy (114 cases), open heart mitral commissurotomy (20 cases), mitral valvuloplasty (5 cases) or aortic commissurotomy (2 cases) reoperated after an average period of 153 +/- 44 months. At reoperation, prosthetic valve replacement of the previously operated valve was systematic and another valvular procedure was also performed in 66 cases. Hospital mortality was 7.8 p. 100. Mortality was high in patients reoperated in functional Class IV of the NYHA classification, after closed heart mitral commissurotomy performed over 10 years before hand. The global mortality rate was 17 p. 100 (average postoperative follow-up of 70 +/- 44 months). The actuarial 5 year survival rate was 85 +/- 6 p. 100 and the 10 year survival was 70 +/- 13 p. 100; NYHA Class IV cardiac failure was a significant poor prognostic factor (p less than 0.05). The prognosis of reoperation after commissurotomy depended mainly on the interval between the relapse of symptoms and reoperation. Group B: comprised 53 patients with valvular prostheses reoperated after an average period of 58 +/- 41 months. The indications of reoperation were prosthetic valve dysfunction (31 cases), perivalvular leak (5 cases), prosthetic valve thrombosis (6 cases), infective endocarditis (7 cases), haemolysis (1 case) and associated valvular disease (10 cases). Reoperation concerned mechanical prostheses in 26 cases and bioprostheses in 24 cases. It consisted in valvular replacement (51 cases) or reinsertion (2 cases). Eight patients underwent second reoperation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anatomo-clinical forms of left endomyocardial fibrosis. Study of 22 surgically treated cases

The authors studied 22 patients with operated left sided endomyocardial fibrosis (EMF). Twenty patients had pure left sided and 2 bilateral (mainly right sided) EMF. The mean age of the patients was 14 years (range 7-51 years). Three clinico-pathological forms of the disease were identified: obliterating, diffuse and limited. The mechanism of associated mitral regurgitation was papillary muscle and/or posterior leaflet involvement. The anterior mitral leaflet was not affected in any of these cases. Echocardiographic diagnosis was possible in 18 cases by M mode recording showing M-shaped septal wall motion. The diagnosis was made in 5 out of 13 patients by 2D echocardiography. This method was satisfactory in the obliterating form of the disease but direct visualisation of the fibrosis in the diffuse and limited forms was very difficult. Catheter studies showed severe pulmonary hypertension in all cases. The presence of a dip-plateau pressure recording was inconstant and was only observed in the obliterating and diffuse forms of EMF. Angiocardiography was characteristic in the obliterating and diffuse forms and enabled an accurate anatomical diagnosis before surgery. The diagnosis of the limited form was difficult and could only be suspected in cases of mitral regurgitation associated with right ventricular EMF or in the presence of parietal recesses. This surgical series does not include cases of EMF limited to the ventricular apex without mitral regurgitation or adiastole, treated medically.     

Conservative surgery of the mitral valve. Immediate and late results

A consecutive series of 130 cases of pure or predominant mitral regurgitation underwent conservative mitral valve surgery between January 1975 and January 1982. The mean age of the patients was 30 +/- 17 years; 25 patients were under 15 years of age. The most common aetiology was rheumatic fever (112/130). Fifty nine patients had associated valvular lesions requiring surgical correction. The patients were divided into 4 groups, according to the surgical technique: Group I: valvular mobilisation (35 cases); Group II: reduction of the amplitude of valvular excursion (48 cases); Group III: mixed valvular mobilisation and reduction of amplitude of excursion (45 cases); Group IV: isolated annuloplasty (2 cases). Hospital mortality was 2.3% (3 patients). Five patients (3.8%) were lost to follow-up. The mean follow-up period was 38 +/- 27 months for the 122 patients followed-up. Seven patients needed reoperation. The long term mortality was 3.1% (4 patients). Four late thromboembolic episodes were observed; they were all transient and regressive in patients with atrial fibrillation. The 7 year actuarial survival rate for the whole series, including hospital mortality, was 92% (1.0 +/- 0.5 patients/year). It was 93.7 +/- 4.9% at 7 years for isolated mitral valvuloplasty and 89.9% +/- 5.6% at 5 years for combined mitro-tricuspid procedures. The actuarial percentage of patients without thromboembolic complications was 91.2% at 7 years with a thromboembolic risk of 1.0 +/- 0.5% patients/year. Eighty eight per cent of patients were not reoperated at 7 years and the reoperation rate was 1.7 +/- 0.7% patients/year.(ABSTRACT TRUNCATED AT 250 WORDS)     

Results of 17 months' surgical experience in the Cardiological Centre of Phnom-Penh (CCPP). The challenge of sanitation in a developing country

The authors report the preliminary results of the first 17 months' experience at the Cardiological Centre of Phnom-Penh (CCPP), set up by the initiative of the "Cha?ne de l'Espoir" organisation. The CCPP has only two operative theatres, 8 intensive care beds and 32 hospital beds. During the 17 months, 1193 patients under 30 years of age were examined. Nine hundred and five had cardiac disease. Three hundred and twenty-two patients underwent surgery for congenital and valvular heart disease. The commonest operated congenital lesions were left-to-right shunts (71%) and tetralogy of Fallot (24%). In the valvular group, 53% had mitral insufficiency, 40% had mitral stenosis and 7% had aortic insufficiency. The selection of patients and surgical strategy depended on the economic constraints specific to developing countries: --strict selection of operable patients: exclusion of severe pulmonary hypertension, left ventricular dysfunction or cachexia and neonates with complex congenital heart disease; --simple, effective and low cost surgical procedures are preferred, repair in the majority of cases, preference given to valvuloplasty over valve replacement. The quality of the results with low morbid-mortality, justifies the creation of such cardiac surgical centres; surgery must be accompanied by strict measures of prevention of endemic rheumatic fever which remains a major public health problem.     

Echocardiography in selecting patients to undergo Carpentier's mitral valvuloplasty

The aim of this study was to confront preoperative echocardiographic data with the anatomic operative findings in patients with mitral insufficiency (MI) undergoing Carpentier's mitral valvuloplasty in order to determine the mechanism(s) of the regurgitation, to classify MI by the echocardiographic changes and to thereby answer the question as to whether echocardiography can identify the patients likely to benefit from this operation. Between February 1985 and November 1987, 66 patients (47 men, 19 women, average age 58 +/- 9 years) with pure MI were referred for surgery with a view to mitral valvuloplasty. This operation was possible in 49 patients (2 of 6 rheumatic MI and 47 of 60 dystrophic MI). The sensitivity of echocardiography was excellent and its specificity very good in diagnosing prolapse of one or the other mitral leaflets. Echocardiography was not as good in distinguishing rupture from elongation of the chordae tendinae and myxoid degeneration from fibro-elastic leaflets. Echocardiography allowed preoperative classification of MI in 4 groups: Group 1 (n = 46) with prolapse of the posterior leaflet; Group 2 (n = 4) with prolapse of the anterior leaflet; Group 3 (n = 8) with prolapse of both mitral leaflets; Group 4 (n = 2) with abnormalities of the mitral annulus alone. Carpentier's valvuloplasty was possible in 43/46 patients in Group 1, 2/4 patients in Group 2, 1/8 patients in Group 3 and 1/2 patients in Group 4. In conclusion, echocardiography is a good tool for selecting patients with dystrophic MI for Carpentier's valvuloplasty.     

Echocardiographic, angiographic and hemodynamic development of surgically treated endomyocardial fibrosis

The echocardiographic, angiographic and haemodynamic evolution of endomyocardial fibrosis (EMF) was assessed in 19 patients: 8 patients with isolated right sided EMF, 3 with predominantly right-sided EMF, and 8 with isolated left sided EMF with mitral regurgitation. Echocardiographic controls were preformed in 16 patients, on average 11.3 months after surgery; angiographic and haemodynamic controls were preformed in 14 patients, on average 14 months after surgery. In isolated or predominantly right-sided EMF, echocardiographic abnormalities of septal motion (paradoxal) disappeared. Although the volume of the right heart chambers decreased, they remained dilated. The syndrome of adiastole disappeared in patients operated early. In left EMF with mitral regurgitation, the M-shaped motion of the septum regressed in the 4 cases in which this abnormality was observed preoperatively. The size of the left heart chambers decreased on post-operative echo and angiographic studies. Left ventricular function returned to normal after surgery. The author underline the differences between right and left-sided EMF with mitral regurgitation. In right-sided EMF the evolution is that of adiastole and surgery should be preformed early because the right ventricular reserve is small. In left-sided EMF with mitral regurgitation, the evolution is that of a valvular lesion with a good postoperative result.     

Factors influencing survival after surgery of pure non-ischemic mitral insufficiency

Uni and multifactorial (Cox) statistical analysis of the results of surgery in a series of 247 patients operated between 1969 and 1988 for pure, non-ischaemic mitral regurgitation was undertaken to determine the factors influencing operative and late mortality. All but 3 cases were adults, average age 51 years, and very symptomatic (75 per cent Class III et IV of NYHA Classification). Dystrophic or degenerative lesions were responsible for 53 per cent of cases of regurgitation whilst rheumatic valvular disease was only observed in 30 per cent of cases. Mitral valve replacement was performed in 137 patients (96 mechanical and 41 bioprostheses) and conservative surgery was possible in 110 cases. There were 12 operative deaths (4.9%); the operative risk increased with age and with the practice of valve replacement. Eleven of the 235 survivors (4.7%) were lost to follow-up. During the follow-up period (average 58 months) there were 37 late deaths of which nearly a half were due to left ventricular dysfunction. Multifactorial analysis identified cardiothoracic ratio and atrial fibrillation as predictive factors of late mortality whilst left ventricular ejection fraction and the type of surgery were related to the development of left ventricular dysfunction. The postoperative left ventricular ejection fraction was significantly lower after valve replacement than after valvuloplasty (11 +/- 13% vs 3 +/- 13%; p less than 0.01). These results are therefore in favour of early correction of severe mitral regurgitation by conservative surgery whenever possible.     

Surgical excision of papillary fibroelastoma for known or potential embolization

BACKGROUND AND AIM OF THE STUDY: Papillary fibroelastoma (PFE) is a rare and histologically benign tumor, but it may have malignant propensity for life-threatening complications. Herein are described four cases of PFE which reflect the clinical diversity of this lesion. The diagnostic and surgical approach utilized is also briefly reviewed. METHODS: The files of four patients with cardiac valvular PFE treated at the authors' center between January 2002 and November 2003 were reviewed. The diagnosis was strongly suggested by echocardiography. Tumors were noted in aortic (n = 2), mitral (n = 1) and tricuspid (n = 1) sites. Indications for surgery were myocardial infarction (both aortic tumors), previous stroke (mitral tumor), and preventive (tricuspid tumor). RESULTS: Surgical excision with a conservative, valve-sparing approach was performed in all cases. For the first aortic tumor, the aortic valve was reconstructed with a patch of autologous pericardium. None of the patients had evidence of valvular regurgitation after excision on intraoperative transesophageal echocardiography, and all had an uneventful recovery. There were no cases of recurrence or regurgitation on follow up echocardiography. CONCLUSION: PFE is an uncommon but increasingly recognized cause of embolic phenomena. Prompt identification allows for surgical excision, which seems to be curative, safe and well-tolerated. A conservative valve-sparing approach is recommended because of the absence of recurrence after total excision.     

Cardiac fibroelastoma. Six operated cases and review of the literature

The authors reviewed the cases of 6 patients operated between September 1994 and February 1999 for a rare benign tumour: papillary fibroelastoma of the heart. These patients, aged 51 +/- 14 years, all have single valvular involvement: mitral (N = 3), aortic (N = 2), tricuspid (N = 1). Five patients were symptomatic: transient ischaemic cerebral events (N = 3) associated with mesenteric infarction requiring ileal resection in 1 case; constituted cerebrovascular accident (N = 1); syncope (N = 1). In this last patient, the fibroelastoma was diagnosed fortuitously on the tricuspid valve. The features and location of the tumours were determined by transoesophageal echocardiography. Surgical treatment in all patients consisted in excising the tumour and preserving the valve. One aortic cusp was reconstructed after excising the tumour with a cryopreserved partial aortic homograft. Peroperative transoesophageal echocardiography confirmed the absence of regurgitation after the procedures in all patients. There were no postoperative complications in any of the cases. No cases of valvular regurgitation or of tumour recurrence were observed during follow-up. Despite the benign histology, cardiac fibroelastomas should be excised because of their embolic complications. Absence of recurrence justifies conservative reconstruction of the affected valve.     

Mitral valve surgery utilizing homografts: early results

BACKGROUND AND AIM OF THE STUDY: Mitral valve repair is superior to prosthetic valve replacement due to preservation of the subvalvular apparatus. We used cryopreserved homografts for mitral valve replacement in selected cases, in whom valve repair would not have been successful. METHODS: Cryopreserved homografts were used in 10 patients (four males, six females; mean age 47 +/- 8 years; range: 27-65 years), for either complete (n = 7) or partial (n = 3) mitral valve replacement. Surgery was indicated due to acute endocarditis (n = 3), stenosis (n = 4) or combined mitral valve disease (n = 3). Transesophageal echocardiography (TEE) was performed to determine correct homograft size; these values were compared with intraoperative measurements of valvular dimensions. The function of the implanted grafts was examined intraoperatively by TEE. Follow up included clinical examination, electrocardiography and echocardiography. RESULTS: All patients survived surgery. Intraoperative TEE revealed mild insufficiency (grade I) in six cases and no insufficiency in four. The papillary muscle-mitral annulus distance was the most reliable preoperative echocardiographic parameter to determine the required homograft. Mean pressure gradients were 3.2 +/- 0.7 mmHg for complete and 2.4 +/- 0.5 mmHg for partial homograft replacement. Sinus rhythm was present in all cases at discharge. Mean follow up was 24 months (range: 6-36 months). At six-month and one-year follow up, the pressure gradients had risen slightly to 3.4 +/- 0.6 mmHg (complete) and 2.8 +/- 0.6 mmHg (partial). To date, four patients had competent grafts, and six presented with an insufficiency (grade I). All patients had normal left ventricular function (EF 65 +/- 6%) at their last follow up; there were no signs of endocarditis during the follow up period. CONCLUSION: Mitral homografts for valve replacement or repair are recommended in selected cases in whom conservative reconstruction techniques are not possible. The avoidance of long-term anticoagulation therapy and preservation of left ventricular geometry are clear advantages to other mitral valve prostheses.     

Rheumatic fever--a review of cases]

OBJECTIVE: To analyse clinical presentation of rheumatic fever (RF), with special emphasis on cardiac involvement, electrocardiographic and echocardiographic findings and the outcome of the cases referred to Maria Pia Children's Hospital from January 1990 to September 1999. METHODS: We retrospectively analyzed the clinical files of all cases referred to pediatric cardiology clinics with the suspicion of acute RF (Group 1) or with rheumatic valvular disease and heart failure (Group 2). In group 1 we studied the following: age and sex distribution, year of diagnosis, presence of Jones criteria treatment and outcome. In group 2 we analysed provenance, age of initial onset of RF, age of cardiology referral, treatment and outcome. RESULTS: Thirteen cases were identified, 8 in groups 1 and 5 in group 2. Group 1 included 3 girls and 2 boys, mean age of 10 years. The diagnosis of RF was based in the presence of 2 major and 1 minor manifestation (4/8), 1 major and 2 minor manifestations (1/8) and chorea in 3 cases associated with clinical carditis in one and subclinical carditis in another. Colour Doppler echocardiography showed pathological mitral regurgitation jet in 6 cases, associated with aortic regurgitation in 2 and dilatation of left ventricle in 3. All were treated with penicillin associated with anti-inflammatory drugs in 5 and haloperidol in 3. Group 2 included 3 girls and 2 boys, mean age 9.56 years. Four were from African countries (Angola and Guinea), and one came from the north of Portugal. The elapsed time between the initial acute attack and cardiology referral varied from 5 months to 3 years. All presented severe mitral insufficiency associated with aortic and/or tricuspid valve lesions, and heart failure. All five underwent valve surgery. The secondary prophylaxis was recommended in every patient. There was a recurrence in a child who had interrupted chemoprophylaxis. The patients from African countries were lost for follow-up. CONCLUSIONS: RF still remains a problem in present times, with serious cardiac sequela in African countries. Colour Doppler echocardiography is a valuable tool for the detection of pathological valvular regurgitation and subclinical carditis if strict criteria are used. The need for appropriate treatment of streptococcal pharyngitis and secondary prophylaxis is emphasized.     

Valvular prolapse in autosomal dominant polycystic kidney]

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.     

Current hospital mortality of aortic valve replacement in octogenarians

BACKGROUND AND AIM OF THE STUDY: The increasing incidence of cardiovascular disease with age, coupled to a constant extension of life expectancy in industrialized countries, is leading to an ever-increasing number of elderly patients being referred for aortic valve replacement (AVR). In light of advances in surgical technology and cardiac protection, the operative mortality and risk factors have been updated in order to specify surgical indications. METHODS: Between January 2000 and December 2004, a total of 442 patients (203 males, 239 females) aged > or =80 years (mean age 82.7 +/- 2.3 years) underwent AVR at the authors' institution. Surgery was either isolated (n = 344) or associated with coronary revascularization (n = 86), mitral valvuloplasty (n = 5) or aortic surgery (n = 7). Seventeen patients had undergone previous cardiac surgery. The EuroScore was calculated for each patient. RESULTS: Overall operative mortality was 7.5% (n = 33). Independent predictive factors of mortality were: aortic insufficiency (30%, p <0.004), NYHA class IV (20.5%, p < 0.001), left and right heart failure (11.5% and 19.4%, p <0.02), chronic renal insufficiency (18.5%, p <0.04), emergency (37.5%, p <0.001, OR = 4.7), left ventricular ejection fraction (21.1%, p <0.004, OR = 0.9), and redo surgery (35.3%, p <0.001, OR = 6). Mortality was also increased in case of associated coronary revascularization (11.6%), mitral or tricuspid surgery (20%) and ascending aorta procedure (25%). CONCLUSION: Patient functional improvement achieved after valve replacement at the cost of a rather low operative mortality justifies considering octogenarians for surgery. However, decisions should be taken on an individual basis. An earlier referral to surgery before the onset of altered cardiac function could lead to further reductions in hospital mortality.