Recommended Criteria for Cardiac Catheterization in Patients with Aortic Valve Disease

Criteria for selection of patients with aortic valve disease for cardiac catheterization are described, based on a study of 81 cases. Children with aortic stenosis warrant catheterization at the time when the clinical diagnosis is made, but in adults this examination may be deferred until symptoms appear or left ventricular hypertrophy is recognized. In patients with pure aortic insufficiency catheterization may be deferred until symptoms appear. When severe stenosis and insufficiency co-exist, the valve is usually heavily calcified. Thirty-seven per cent of patients with aortic valve disease have co-existing mitral lesions and these patients are usually women, are fibrillating and, as a rule, have atrial enlargement in contrast to those with aortic valve disease only. On rare occasions, patients with mitral valve disease have clinically silent but angiographically demonstrable aortic insufficiency; therefore, aortography should precede open-heart correction of a mitral lesion so as to detect minor degrees of aortic insufficiency.     

Left heart catheterization; the clinical importance of findings

Left heart catheterization using the transbronchial route to obtain pressures in the left atrium and left ventricle was used successfully in 29 cases with no mortality or morbidity. It was found to be useful in differentiating between mitral stenosis and mitral insufficiency, as well as determining the amount of aortic stenosis present when there was involvement of the aortic valve. The technique was also helpful in determining which is the predominant lesion when there is a disease of the aortic and mitral valves. In two patients in a series of 29, data obtained by left heart catheterization forestalled operation on the basis of a mistaken diagnosis of mitral stenosis when actually no mitral valvular disease was present. In another eight patients, the predominant lesion was found to be mitral stenosis rather than mitral insufficiency as it was thought to be before catheterization. In two patients, who had only systolic murmurs, catheterization revealed mitral stenosis rather than mitral insufficiency. In four patients who were thought to have mixed valvular disease, left heart catheterization showed only aortic valvular disease.     

LEFT HEART CATHETERIZATION—The Clinical Importance of Findings

Left heart catheterization using the transbronchial route to obtain pressures in the left atrium and left ventricle was used successfully in 29 cases with no mortality or morbidity. It was found to be useful in differentiating between mitral stenosis and mitral insufficiency, as well as determining the amount of aortic stenosis present when there was involvement of the aortic valve. The technique was also helpful in determining which is the predominant lesion when there is a disease of the aortic and mitral valves.In two patients in a series of 29, data obtained by left heart catheterization forestalled operation on the basis of a mistaken diagnosis of mitral stenosis when actually no mitral valvular disease was present. In another eight patients, the predominant lesion was found to be mitral stenosis rather than mitral insufficiency as it was thought to be before catheterization. In two patients, who had only systolic murmurs, catheterization revealed mitral stenosis rather than mitral insufficiency. In four patients who were thought to have mixed valvular disease, left heart catheterization showed only aortic valvular disease.     

Occult aortic stenosis as cause of intractable heart failure

During a three-year period 10 patients with critical aortic stenosis were referred to a cardiac referral centre with symptoms and signs of intractable cardiac failure and low cardiac output. In nine patients the correct diagnosis was not suspected at the referring hospital, and in the remaining patient the true severity of the aortic stenosis was not appreciated and cardiomyopathy was suggested as an additional diagnosis. The most common referral diagnoses were severe mitral regurgitation (four patients), congestive cardiomyopathy (two patients), or both (three patients). Only two patients had soft ejection systolic murmurs at the base of the heart radiating into the neck, and such a murmur appeared in a third patient during medical treatment. The carotid pulses were of small volume but the characteristic slow-rising, anacrotic nature of the pulse could not be appreciated clinically. The diagnosis was suspected in nine patients because of aortic valve calcification detected by lateral chest x-ray examination in seven patients and by x-ray screening of the heart in two, and because of abnormal aortic valve echoes in the echocardiogram of all five patients in whom the aortic valve could be seen. Eight patients underwent aortic valve replacement despite seemingly poor preoperative left ventricular function. Three patients died, of whom two had severe coexistent coronary artery disease. The five survivors all returned to normal lives and needed little or no medication.Critical aortic stenosis should be actively sought in patients with severe heart failure of unknown cause since surgery may enable them to resume their normal lives.     

Pregnancy in women with prosthetic heart valves

Pregnancy in women with mechanical valve prostheses has a high maternal complication rate including valve thrombosis and death. Coumarin derivatives are relatively safe for the mother with a lower incidence of valve thrombosis than un-fractionated and low-molecular-weight heparin, but carry the risk of embryopathy, which is probably dose-dependent. The different anticoagulation regimens are discussed in this review. When valve thrombosis occurs during pregnancy, thrombolysis is the preferable therapeutic option. Bioprostheses have a more favourable pregnancy outcome than mechanical prostheses but due to the high re-operation rate in young women they do not constitute the ideal alternative. When women with native valve stenosis need pre-pregnancy intervention, mitral balloon valvuloplasty is the best option in mitral stenosis, while the Ross operation or homograft implantation may be the preferable surgical regimen in aortic stenosis. (Neth Heart J 2008;16:406-11.)     

Quality of life among patients with severe aortic stenosis

BackgroundThe disease burden of patients with severe aortic stenosis is not often explored, while the incidence is increasing and many patients who have an indication for aortic valve replacement are not referred for surgery. We studied the quality of life of 191 patients with severe aortic stenosis, hypothesising that symptomatic patients have a far worse quality of life than the general population, which could enforce the indication for surgery.MethodsThe SF-36v2 Health Survey was completed by 191 consecutive patients with symptomatic or asymptomatic severe aortic stenosis.ResultsAsymptomatic patients (n = 59) had health scores comparable with the general Dutch population but symptomatic patients (n = 132) scored significantly lower across different age categories. Physical functioning, general health and vitality were impaired, as well as social functioning and emotional well-being. There was no relation between degree of stenosis and physical or mental health scores.ConclusionsBoth physical and emotional problems have a major impact on normal daily life and social functioning of symptomatic patients with severe aortic stenosis, regardless of age. If the aortic stenosis is above the ‘severe’ threshold, the degree of stenosis does not predict disease burden. These results encourage to reconsider a conservative approach in symptomatic patients with severe aortic stenosis. Using the SF-36v2 Health Survey together with this study, an individual patient’s quality of life profile can be assessed and compared with the patient group or with the general population. This can assist in decision making for the individual patient.     

Ventriculo-atrial gradient due to first degree atrio-ventricular block: a case report

Background

Isolated, asymptomatic first degree AV block with narrow QRS has not prognostic significance and is not usually treated with pacemaker implantation. In some cases, yet, loss of AV synchrony because of a marked prolongation of the PR interval may cause important hemodynamic alterations, with subsequent symptoms of heart failure. Indeed, AV synchrony is crucial when atrial systole, the "atrial kick", contributes in a major way to left ventricular filling, as in case of reduced left ventricular compliance because of aging or concomitant structural heart disease.

Case presentation

We performed a trans-septal left atrium catheterization aimed at evaluating the entity of a mitral valve stenosis in a 72-year-old woman with a marked first-degree AV block, a known moderate aortic stenosis and NYHA class III symptoms of functional deterioration. We occurred in a deep alteration in cardiac hemodynamics consisting in an end-diastolic ventriculo-atrial gradient without any evidence of mitral stenosis. The patient had a substantial improvement in echocardiographic parameters and in her symptoms of heart failure after permanent pacemaker implantation with physiological AV delay.

Conclusion

We conclude that if a marked first degree AV block is associated to instrumental signs or symptoms of heart failure, the restoration of an optimal AV synchrony, achieved with dual-chamber pacing, may represent a reasonable therapeutic option leading to a consequent clinical improvement.     

Percutaneous valve procedures: present and future

Percutaneous mitral commissurotomy and aortic valvuloplasty have been being performed since the mid-1980s. Balloon commissurotomy has been used in thousands of cases worldwide and it provides good short- and long-term results in a wide range of patients. It has virtually replaced surgical commissurotomy in the treatment of mitral stenosis. In contrast, percutaneous aortic valvuloplasty is almost abandoned worldwide due to its lack of efficacy and the risks involved. The new techniques of percutaneous valve intervention: aortic valve replacement and mitral valve repair are at an early stage: the first in-man applications of these fledgling techniques started in 2002. Preliminary series show that they are feasible; however, they need to be further evaluated in comparison with contemporary treatment to assess accurately how efficient they are and the risks involved. Currently potential applications concern high-risk patients, however, in the future, after thorough evaluation, this may be extended to others. Thus, percutaneous interventions already play an important role in treatment of valvular heart disease, a role that seems set for future expansion.     

Book Reviews

The findings in and experiences with 19 consecutive patients subjected to open mitral valve surgery are described. All patients underwent a right-heart catheterization. In order to exclude multivalvular heart disease, a left-heart catheterization was performed in 10 patients and angiographic studies of the aortic valve area in 12. Pulmonary function studies were performed on 11 patients. The FEV (0.75 sec.) and the MMFR were found to correlate well with existing pulmonary reserves.Fourteen of the 19 patients subjected to open mitral valve surgery survived the operation and have been followed up for three months to 2½ years. Four patients had a mitral valvuloplasty. Three of these four have deteriorated and will require a valvular replacement. Ten of 15 patients subjected to a mitral valve replacement are alive; five of these 10 have had signs and symptoms indicating peripheral embolization. Mitral valvuloplasty is preferable to a valvular replacement as far as embolic complications are concerned, while valvular replacements result in a more perfect and lasting hemodynamic repair. The high incidence of peripheral embolization following valvular replacements focuses attention on the need for improved mitral valve prostheses.     

Mitral stenosis with posterior diastolic movement of posterior leaflet.

The echocardiographic diagnosis of mitral stenosis depends in part on the demonstration of abnormal posterior leaflet movement to distinguish it from other conditions that similarly affect anterior leaflet motion. In mitral stenosis the posterior leaflet has been shown to move anteriorly in diastole rather than in the normal posterior direction. A patient presented with clinical evidence of moderate mitral stenosis. The anterior leaflet echo was typical but the posterior leaflet showed posterior diastolic movement. At catheterization moderate mitral stenosis was confirmed. To our knowledge this is the first report of the echocardiographic demonstration of posterior diastolic movement of the posterior mitral leaflet in documented mitral stenosis.     

Valvular heart disease; the role of cardiac catheterization in preoperative evaluation

Cardiac catheterization studies performed in research laboratories showed that advanced mitral stenosis is associated with a characteristic dynamic pattern which is reversible by mitral valvulotomy. In the process of the selection of patients for mitral valvulotomy, occasionally there are instances in which a decision cannot be reached on the basis of ordinary clinical methods of examination. In some such cases cardiac catheterization may be of decisive value by demonstrating, or by failing to demonstrate, the dynamic pattern of mitral stenosis. Cases in which this diagnostic procedure is most often helpful are those of mild mitral stenosis and those in which there are combined valvular defects.     

Exuberant accessory mitral valve tissue with possible true parachute mitral valve: a case report

ABSTRACT: INTRODUCTION: A parachute mitral valve is defined as a unifocal attachment of mitral valve chordae tendineae independent of the number of papillary muscles. Data from the literature suggests that the valve can be distinguished on the basis of morphological features as either a parachute-like asymmetrical mitral valve or a true parachute mitral valve. A parachute-like asymmetrical mitral valve has two papillary muscles; one is elongated and located higher in the left ventricle. A true parachute mitral valve has a single papillary muscle that receives all chordae, as was present in our patient. Patients with parachute mitral valves during childhood have multilevel left-side heart obstructions, with poor outcomes without operative treatment. The finding of a parachute mitral valve in an adult patient is extremely rare, especially as an isolated lesion. In adults, the unifocal attachment of the chordae results in a slightly restricted valve opening and, more frequently, valvular regurgitation. CASE PRESENTATION: A 40-year-old Caucasian female patient was admitted to a primary care physician due to her recent symptoms of heart palpitation and chest discomfort on effort. Transthoracic echocardiography showed chordae tendineae which were elongated and formed an unusual net shape penetrating into left ventricle cavity. The parasternal short axis view of her left ventricle showed a single papillary muscle positioned on one side in the posteromedial commissure receiving all chordae. Her mitral valve orifice was slightly eccentric and the chordae were converting into a single papillary muscle. Mitral regurgitation was present and it was graded as moderate to severe. Her left atrium was enlarged. There were no signs of mitral stenosis or a subvalvular ring. She did not have a bicuspid aortic valve or coarctation of the ascending aorta. The dimensions and systolic function of her left ventricle were normal. Our patient had a normal body habitus, without signs of heart failure. Her functional status was graded as class I according to the New York Heart Association grading. CONCLUSIONS: A recently published review found that, in the last several decades, there have been only nine adult patients with parachute mitral valve disease reported, of which five had the same morphological characteristics as our patient. This case presentation should encourage doctors, especially those involved in echocardiography, to contribute their own experience, knowledge and research in parachute mitral valve disease to enrich statistical and epidemiologic databases and aid clinicians in getting acquainted with this rare disease.     

MITRAL VALVE PROLAPSE AND CONGENITAL PYLORIC STENOSIS IN IDENTICAL TWINS

Both congenital pyloric stenosis and the mitral valve prolapse syndrome are reported to have a familial incidence. Although mitral valve prolapse has been documented in twins, only one case has received echocardiographic confirmation. The present account describes typical symptomatic mitral valve prolapse in identical twins, both of whom had undergone surgery during infancy to correct congenital pyloric stenosis.     

The Estimation of Aortic Insufficiency

Thirty patients were investigated to determine the degree of aortic insufficiency present in them, using the test of Braunwald and Morrow in which indicator dye is injected at descending levels in the aorta and regurgitant dye is recorded by oximetry at the right ear. In the presence of aortic insufficiency regurgitant dye was demonstrated from as low as the second lumbar level, while in normal subjects none was demonstrated on injection distal to the fifth thoracic level. In the older patients the level was more proximal than in the younger for similar regurgitant flows. In most patients the test correlated well with the clinical impression of the severity of the lesion. However, tachycardia, bradycardia a reduced forward flow secondary to failure, aortic stenosis, or mitral stenosis or insufficiency will modify the level from which regurgitation occurs. Estimation of forward flow would permit estimation of the percentage regurgitation and allow a more accurate assessment of aortic insufficiency. Preoperative and postoperative estimation permitted objective assessment of successful corrective surgery.     

Rheumatic fever and rheumatic heart disease; incidence in California

The statistics quoted in this and other published reports appear to substantiate the impression that rheumatic fever in California, although still an important public health problem which varies widely from one locality to another, is of lower incidence and perhaps of more benign character than in most other parts of the United States. It also appears that in California aortic insufficiency may be of higher relative incidence and occurs more frequently as a clinically diagnosable sequel of rheumatic fever than does classical mitral stenosis. Congenital defects of the heart constitute a large proportion of the cases of organic heart disease in children and young adults in this state.     

RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE—Incidence in California

The statistics quoted in this and other published reports appear to substantiate the impression that rheumatic fever in California, although still an important public health problem which varies widely from one locality to another, is of lower incidence and perhaps of more benign character than in most other parts of the United States. It also appears that in California aortic insufficiency may be of higher relative incidence and occurs more frequently as a clinically diagnosable sequel of rheumatic fever than does classical mitral stenosis. Congenital defects of the heart constitute a large proportion of the cases of organic heart disease in children and young adults in this state.     

Undiagnosed hypertrophic obstructive cardiomyopathy during transcatheter aortic valve replacement: a case report

Background

Transcatheter aortic valve replacement is indicated for severe symptomatic aortic stenosis in patients who have a very high or prohibitive surgical risk as assessed pre-procedurally by the Society of Thoracic Surgery Risk Score, EuroSCORE (II), frailty testing, and other predictors. When combined with another left ventricular outflow tract obstruction, careful consideration must be taken prior to proceeding with transcatheter aortic valve replacement because an additional masked left ventricular outflow tract pathology can lead to challenging hemodynamics in the peri-deployment phase, as reported in this case.

Case presentation

A 56-year-old Caucasian man with multiple comorbidities and severe aortic stenosis underwent transcatheter aortic valve replacement under monitored anesthesia care. During the deployment phase, he developed dyspnea that progressed to pulmonary edema requiring emergent conversion to general anesthesia, orotracheal intubation, acute respiratory distress syndrome-type ventilation, and vasopressor medications. Intraoperative transesophageal echocardiography was performed and hypertrophic obstructive cardiomyopathy with systolic anterior motion of the mitral valve was discovered as an underlying pathology, undetected on preoperative imaging. After treatment with beta blockers, fluid resuscitation, and alpha-1 agonists, he stabilized and was eventually discharged from our hospital without any lasting sequelae.

Conclusions

Patients with aortic stenosis most often develop symmetric hypertrophy; however, a small subset has asymmetric septal hypertrophy leading to left ventricular outflow tract obstruction. In cases of severe aortic stenosis, however, evidence of left ventricular outflow tract obstruction via both symptoms and echocardiographic findings may be minimized due to extremely high afterload on the left ventricle. Diagnosing a left ventricular outflow tract obstruction as the cause of hemodynamic instability during transcatheter aortic valve replacement, in the absence of abnormal findings on echocardiogram preoperatively, requires a high index of clinical suspicion. The management of acute onset left ventricular outflow tract obstruction intraoperatively consists primarily of medical therapy, including rate control, adequate volume resuscitation, and avoidance of inotropes. With persistently elevated gradients, interventional treatments may be considered.

     

同期施行冠状动脉搭桥和心脏瓣膜手术的体外循环

目的总结和分析同期施行冠状动脉搭桥和心脏瓣膜手术的体外循环方法。方法125例患者分为3组:M组(冠脉病变及二尖瓣病变)75例,A组(冠脉病变及主动脉瓣病变)34例,D组(冠脉病变及二尖瓣和主动脉瓣病变)16例。心肌保护采用4:1冷含血停搏液,应用单纯顺灌、顺灌逆灌结合、顺灌桥灌结合、顺逆灌和桥灌结合技术。结果术中转流平稳,血流动力学稳定,监测指标均在正常范围,无手术死亡。结论同期施行冠状动脉搭桥和心脏瓣膜手术,术中良好的心肌保护方法和合理的体外循环灌注是保证手术顺利成功的重要因素。     

经桡动脉心导管术诊断和治疗冠状动脉疾病的临床研究

目的:观察经桡动脉介入诊治冠心病的安全性和有效性。方法:对1503例患者进行了经桡动脉途径介入诊治,其中包括心绞痛患者1218例,心梗患者268例,二尖瓣和/或动脉瓣手术前对冠状动脉进行解剖学评估17例,观察患者手术安全性、成功率以及与经桡动脉手术相关的并发症发病率。结果:介入治疗成功率为93.1%(1400/1503),手术失败的主要原因为桡动脉穿刺失败(39例),严重桡动脉痉挛(42例),右锁骨下动脉不同程度的弯曲(18例),近端严重狭窄(14例)。术后有14例患者出现严重并发症(瞬时缺血性病变),60例出现有症状的窦性心动过缓,43例出现静脉血栓,77例出现血肿,88例出现桡动脉梗阻。结论:经桡动脉途径是一种安全、有效、患者更容易接受的冠心病介入诊治方法。     

Fabry disease: recent advances in pathology, diagnosis, treatment and monitoring

Patent arterial duct (PAD) is a congenital heart abnormality defined as persistent patency in term infants older than three months. Isolated PAD is found in around 1 in 2000 full term infants. A higher prevalence is found in preterm infants, especially those with low birth weight. The female to male ratio is 2:1. Most patients are asymptomatic when the duct is small. With a moderate-to-large duct, a characteristic continuous heart murmur (loudest in the left upper chest or infraclavicular area) is typical. The precordium may be hyperactive and peripheral pulses are bounding with a wide pulse pressure. Tachycardia, exertional dyspnoea, laboured breathing, fatigue or poor growth are common. Large shunts may lead to failure to thrive, recurrent infection of the upper respiratory tract and congestive heart failure. In the majority of cases of PAD there is no identifiable cause. Persistence of the duct is associated with chromosomal aberrations, asphyxia at birth, birth at high altitude and congenital rubella. Occasional cases are associated with specific genetic defects (trisomy 21 and 18, and the Rubinstein-Taybi and CHARGE syndromes). Familial occurrence of PAD is uncommon and the usual mechanism of inheritance is considered to be polygenic with a recurrence risk of 3%. Rare families with isolated PAD have been described in which the mode of inheritance appears to be dominant or recessive. Familial incidence of PAD has also been linked to Char syndrome, familial thoracic aortic aneurysm/dissection associated with patent arterial duct, and familial patent arterial duct and bicuspid aortic valve associated with hand abnormalities. Diagnosis is based on clinical examination and confirmed with transthoracic echocardiography. Assessment of ductal blood flow can be made using colour flow mapping and pulsed wave Doppler. Antenatal diagnosis is not possible, as PAD is a normal structure during antenatal life. Conditions with signs and symptoms of pulmonary overcirculation secondary to a left-to-right shunt must be excluded. Coronary, systemic and pulmonary arteriovenous fistula, peripheral pulmonary stenosis and ventricular septal defect with aortic regurgitation and collateral vessels must be differentiated from PAD on echocardiogram. In preterm infants with symptomatic heart failure secondary to PAD, treatment may be achieved by surgical ligation or with medical therapy blocking prostaglandin synthesis (indomethacin or ibuprofen). Transcatheter closure of the duct is usually indicated in older children. PAD in preterm and low birth weight infants is associated with significant co-morbidity and mortality due to haemodynamic instability. Asymptomatic patients with a small duct have a normal vital prognosis but have a lifetime risk of endocarditis. Patients with moderate-to-large ducts with significant haemodynamic alterations may develop irreversible changes to pulmonary vascularity and pulmonary hypertension.