Extracardiac Diseases Research Articles

Platypnea-Orthodeoxia Syndrome Related to an Aortic Aneurysm Combined With an Aneurysm of the Atrial Septum

We report the case of a 71-year-old man bearing a severe right-to-left shunt through a patent foramen ovale in the absence of elevated right-sided heart or pulmonary artery pressures. He presented with platypnea-orthodeoxia syndrome, but he had no pulmonary or extracardiac diseases that are known to be associated with this syndrome. Chest radiography showed a bulky aneurysm of the thoracic aorta. A peripheral contrast transesophageal echocardiography demonstrated a large right-to-left shunt through a patent foramen ovale. In addition, the atrial septum was severely deformed by an aneurysm including this patent foramen ovale. We hypothesized that the opening of the foramen ovale was the result of a mechanical deformation of the atrial septum by two contributing factors: the aneurysm of the thoracic aorta and the aneurysm of the septum itself.

Will Noninvasive Techniques Replace Coronary Angiography?

Electron beam computed tomography (EBCT) and magnetic resonance imaging (MRI) represent modern noninvasive imaging technologies. MRI in particular has improved diagnosis of many extracardiac diseases. It is claimed that these technologies are superior to the established technique of coronary angiography (CA) for diagnosis of coronary artery disease (CAD) because they are easily repeatable, eliminating the risk of an invasive procedure, and potentially cost saving. Both EBCT and MRI have produced excellent images of proximal coronary arteries. However, their imaging precision is far from that of CA, especially for distal artery segments and the left circumflex and right coronary arteries. An improved image quality probably will be achieved in the future. However, for the next several years CA is essential for diagnosis of CAD and planning of therapy. In selected patients, MRI will provide additional information to angiographic and echocardiographic data (e.g., for assessment of coronary flow, myocardial viability, and morphology of extracardiac vessels). For reasons of cost containment, in many patients with CAD the simultaneous procedure of diagnostic CA and therapeutic angioplasty seems more reasonable than the additional expensive performance of EBCT and MRI.

Echo‐Doppler left ventricular filling abnormalities in patients with rheumatoid arthritis without clinically evident cardiovascular disease

Our investigation aimed at verifying diastolic abnormalities in rheumatoid patients, without clinically evident cardiovascular disease and other confounding complaints, by using pulsed Doppler examination of transmitral blood flow. We selected 40 patients fulfilling revised American Rheumatism Association (ARA) criteria for the diagnosis of rheumatoid arthritis having no symptoms of cardiac disease or clinical findings of other extracardiac diseases. We also studied 40 rheumatoid-matched healthy volunteers as a control group. An echocardiographic examination was carried out on each subject. Left ventricular structural and functional measurements were obtained. Interventricular, septal thickness and left ventricular mass index were significantly higher in rheumatoid patients than in the control group. We also found in rheumatoid patients higher mean values of peak A velocity and A/E ratio. When multiple linear regression analysis was performed on the data of rheumatoid patients we found an independent relationship only between A/E ratio and left ventricular mass. In conclusion, our results confirm diastolic abnormalities in rheumatoid patients and point out that these abnormalities also affect echo-Doppler parameters of left ventricular filling. Moreover, further analysis of our data may suggest the possibility that structural left ventricle changes could be responsible for left ventricular filling impairment.

Fetal and transient neonatal right heart dilatation with severe tricuspid valve insufficiency in association with abnormally S‐shaped kinking of the ductus arteriosus

Alterations of the ductus arteriosus, e.g. occlusion, constriction or aneurysm, can influence the fetal and neonatal circulation. A case of fetal right atrial and ventricular dilatation, severe tricuspid valve insufficiency and transient pulmonary valve regurgitation at 32 weeks of gestation is reported. Structural heart defects and extracardiac diseases (e.g. high output cardiac failure in arteriovenous shunts and anemia) were excluded by Doppler echocardiography and fetal blood sampling. An abnormally S-shaped kinking of the ductus arteriosus with increased systolic and diastolic flow velocities in the distal part was demonstrated. At 35 weeks' gestation, Cesarean section was performed, because of increased tricuspid valve insufficiency, and a healthy boy was born. After birth, right heart dilatation and tricuspid valve insufficiency decreased. The cardiac changes in relation to the anatomical abnormality of the ductus arteriosus are discussed.

Cardiac involvement in rheumatoid arthritis: evidence of silent heart disease.

Rheumatoid arthritis (RA) is a systemic disease involving many organ systems and is frequently accompanied by cardiac alterations. However, there is considerable disagreement concerning the cardiac abnormalities found in patients with RA. The purpose of our investigation was to determine, by a non-invasive method such as echocardiography, the nature and extent of cardiac involvement in RA patients with no symptoms of cardiac disease, in comparison with a control sample. We selected 35 patients affected by rheumatoid arthritis (five men, 30 women), aged 51 +/- 11 years. No patient had either symptoms of cardiac disease or extra cardiac complaint. As a control group we studied 52 volunteers, aged 51 +/- 12 years, randomly selected among a larger group of subjects with no symptoms, signs and/or clinical findings of extra cardiac diseases. All were in sinus rhythm and without any cardiac symptom. Standard two-dimensional, M-mode and Doppler echocardiographic examination was carried out on each subject. In RA patients we found a higher prevalence of several abnormalities. We found no statistically significant differences between the groups of RA patients based on the stage and duration of disease. We found no correlation between cardiac abnormalities and inflammatory indices or drug therapy. At least three alterations seem to be typical of RA patients in the absence of any symptom of cardiac disease: (1) posterior pericardial effusion, (2) aortic root alterations and (3) valvular thickening. The prevalence of MVP is controversial and needs further investigation. These alterations are variously combined in each patient, and for this reason we think that it is possible to represent such a heart involvement as 'silent rheumatoid heart disease'. Moreover the knowledge of the presence of unrecognised cardiac abnormalities can be very important for the correct assessment and management of the RA patient.

Successful repair of a ruptured abdominal aortic aneurysm in a cardiac transplant patient

The improvement in survival rates for heart transplant recipients (HTRs) has increased their risk of developing extracardiac diseases such as abdominal aortic aneurysms (AAAs). The purposes of this study were to evaluate the prevalence and to describe the clinical features and natural history of AAA in HTRs.A retrospective review of all patients (375) who underwent heart transplantation (HT) at our center over a 32-year period (1983–2015) was carried out.We identified 20 patients (5.3%) with AAA. All but one patient were male (95%), and most of them (80%) had a history of ischemic heart disease (IHD) prior to transplantation. The mean age of the patients with AAA at transplant was 57.2 ± 7.3 years (range: 42–62 years). Seven of the 20 patients with AAA already had an AAA (30–55 mm) prior to transplantation. The average aneurysm size at the time of diagnosis was 40.9 ± 9.6 mm, and the average patient age at the time of diagnosis was 62.2 ± 8.3 years. The mean linear expansion rate was 10.6 ± 2.12 mm/y, and the exponential expansion rate was 0.220 ± 0.040 year−1, respectively. The median follow-up time was 5.4 years (range 0.1–27.4 years). The median survival was 143 months (95% confidence interval (CI) 65 to 180 months) for the 20 HTRs with AAA and 68.8 months (95% CI 46 to 88 months) for the other HTRs.The natural history of AAA in HTR is characterized by an increased expansion rate. Male HTR with end-stage IHD are particularly at risk and should be closely followed-up after HT.

Primary surgical closure of large ventricular septal defects in small infants

Herein, a policy of primary surgical closure of large ventricular septal defects in infants is reviewed. Fortyeight infants met criteria for inclusion in the study, and were divided into two groups based on weight: group 1 infants weighed 4 kg or less (n = 23), and group 2 infants weighed more than 4 kg (n = 25). Both groups had similar variation in ventricular septal defect location (paramembranous versus muscular) and number (single versus multiple), as well as incidence of major associated extracardiac diseases. No early deaths occurred in group 1, compared with 1 infant (4%) in group 2. Major complications occurred similarly in both groups (9% versus 12%). There were two late deaths in group 1 (9%) and none in group 2. No surviving patients have required a second ventricular septal defect operation, and the majority no longer receive anticongestive therapies. These results indicate that primary surgical closure of large ventricular septal defects, even multiple muscular defects, can be performed in very small infants with no difference in mortality or serious complication rates compared with larger infants. Protracted medical efforts to achieve larger size before primary repair and palliative pulmonary artery banding are not necessary.

Postoperative pathology of complete atrioventricular defects

Necropsy data on 15 patients, who died after operations for complete atrioventricular defect (CAVD), were reviewed in order to analyze the possible determinants of death. Two infants had undergone a palliative procedure--pulmonary artery banding (Group I); three patients had had prosthetic replacement of one or both atrioventricular (AV) valves (Groups II); and the remaining 10 had had conservative repair with plastic reconstruction of the AV valves (Group III). The most notable pathological findings related to death were as follows: Group I, left ventricular hypoplasia in both infants; Group II, prosthetic dysfunction in all patients; Group III, extracardiac diseases in two-patients and cardiac lesions in six. In Group III severe pulmonary vascular obstructive disease (PVOD) was observed as an isolated finding in two patients and in combination with residual intracardiac anomalies in another two. Analysis of the incidence of PVOD demonstrated that irreversible changes may occur within the first year of life in babies with Down's syndrome, and that these changes well correlate with the calculated pulmonary vascular resistance (PVR) values. From the results of this study we have reached the following conclusions: (1) Prosthetic valve replacement has to be avoided because of a high incidence of prosthesis-related complications and the effectiveness of conservative repair; (2) residual untreated anomalies may affect the outcome of operation and should be accurately recognized and weighted preoperatively; (3) PVOD can occur in CAVD even before the first year of life and can be reliably assessed by the measurement of PVR. High degrees of PVOD frequently interfere with the surgical success, particularly when residual intracardiac anomalies are present. Therefore, we suggest that surgical correction be planned, when indicated, during the first 6 months of life.

Systolic triple rhythms

1. 1. The records of sixty-four cases of systolic triple rhythm have been reviewed. The presence or absence of cardiovascular disease and the various characteristics of these sounds were evaluated. 2. 2. Systolic triple rhythms are probably more frequently present than generally suspected and may be heard in a wide variety of cardiac and extracardiac diseases. 3. 3. Early systolic clicks are ejection sounds and are found in patients with congenital and acquired lesions of the semilunar valves and great vessels and are usually associated with a murmur. 4. 4. Mid- or late systolic sounds may or may not be associated with a murmur and are usually not observed in patients with cardiovascular disease. 5. 5. With a thorough understanding of the characteristics of systolic sounds, their significance may be correctly interpreted.

Reversible forms of heart disease.

For many years the diagnosis of heart disease connoted a life of invalidism with probable death by virtue of the affliction. In recent years, however, the marked advances in medical science have not only enabled the heart patient to live a more useful life but also have shown that many forms of heart disease are entirely curable. It is important to remember that a normal heart or one that is only slightly diseased may show symptoms that are solely the expression of some extracardiac disease, and that this extracardiac condition may manifest itself entirely or largely in the cardiovascular system. As a result, the cardiac symptoms may be so marked that they completely overshadow the underlying extracardiac disease. As a result the patient is often considered a cardiac invalid, whereas recognition of the effect of the extracardiac disease with treatment directed to the extracardiac condition usually brings about a reversibility in the cardiovascular symptoms. Failure on our part to recogni e that many extracardiac diseases are expressed clinically through the cardiovascular system has led to unnecessary invalidism and even death. In every cardiac patient we must constantly be aware of the possibility that the heart itself may not be actually diseased but that rather it is acting secondarily to other conditions in the body. It is only by recognizing these extracardiac factors before they produce structural changes in the heart muscle that the treatment of the extracardiac disease may be instituted sufficiently early for the patient to be cured. It is the purpose of this paper to point out in a general way some conditions that are manifested entirely or largely through the cardiovascular system, with the result that a normal or a previously damaged heart may be precipitated into actual cardiac failure. It is apparent that all of the diseases that may produce such changes cannot be listed in the space permitted, and under these circumstances only a brief mention will be made of the various illnesses that can affect the cardiovascular system. We are familiar with the symptoms presented by the markedly obese patient. He frequently complains of dyspnea, palpitation and precordial distress. Physical examination may often reveal an