Quiz case of the month. Pulmonary arteriovenous malformation.
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Arteriovenous Malformations↗
Biomedical subjects
W Budts
Publications and source records attributed to W Budts.
Follow-up of patients with previous treatment for coarctation of the thoracic aorta: comparison between contrast-enhanced MR angiography and fast spin-echo MR imaging.
Regular follow-up is required in patients with previous intervention for coarctation of the aorta to detect recoarctation or aneurysm formation. In this study we describe the findings encountered on routine follow-up exams and we compare the use of contrast-enhanced 3D MR angiography (CE MRA) with fast spin-echo MRI (FSE) to study the thoracic aorta after previous intervention. In 51 consecutive patients previously treated for aortic coarctation, 74 MR studies of the thoracic aorta were performed during a 2-year period using CE MRA and FSE MRI. The thoracic aorta was evaluated for abnormalities of course, caliber, shape, and pathology of side branches. The CE MRA and FSE MRI studies were evaluated side by side by consensus of two reviewers evaluating which MR technique depicted the abnormalities of the thoracic aorta the best. Of 74 exams, six clinically important abnormalities were found: four aneurysms and two restenoses. Two small pseudoaneurysms were missed on the FSE studies. Contrast-enhanced MRA was judged to visualize aortic abnormalities better than FSE (47 of 74 MR studies) especially for the transverse aortic arch, coarctation site, left subclavian artery, and aortic arch configuration. For the ascending aorta and distal descending aorta, CE MRA and FSE performed equally well. Aortic diameters measured at four levels in the first 18 MRI studies showed no significant differences in diameter when measured by FSE or CE MRA (p = not significant). Clinically important abnormalities, such as aneurysm formation and restenosis, can be present years after treatment for aortic coarctation. In the regular follow-up of these patients, CE MRA may provide additional diagnostic information compared with FSE and should be included as part of the routine exam.
Adolescent↗
A 72 year old woman with ALCAPA.
ALCAPA syndrome (anomalous origin of the left coronary artery from the pulmonary artery), which causes the left coronary artery to grow with an anomalous origin from the pulmonary artery, is a rare disease which may result in myocardial infarction, congestive heart failure, and sometimes death during the early infantile period. A 72 year old woman with ALCAPA syndrome is presented. The asymptomatic patient presented with a cardiac murmur which was discovered during a routine check up for a gynaecological intervention. Coronary cineangiography established the diagnosis. Although surgical correction is the usual treatment for such cases, medical treatment was preferred for this patient because she was asymptomatic without clinical signs of heart failure.
Aged↗
Failure of medicine: evolution of an atrial septal defect to an Eisenmenger syndrome.
The atrial septal defect (ASD) is the most commonly diagnosed congenital defect in adults and has a prevalence of 7.5% of all congenital cardiac anomalies. Less invasive imaging techniques, especially transthoracic and transoesophageal echocardiography, provide more accurate diagnostics, resulting in earlier diagnosis and treatment. Despite these opportunities in high-tech countries, medicine may still fail in detecting initially correctable cardiac anomalies. We present a case of 41-year-old woman with an abnormal murmur at childhood that disappeared with time due to the development of an Eisenmenger syndrome. The importance of a complete haemodynamic evaluation in this patient is illustrated.
Adult↗
Pulmonary stenosis caused by extrinsic compression of an aortic pseudoaneurysm of a composite aortic graft.
Pulmonic stenosis and stenosis of the right ventricular outflow tract related to extrinsic compression have been described in patients with tumors, in a patient with a pericardial cyst, and in patients with vascular abnormalities as an unruptured sinus of Valsalva aneurysm, a giant coronary artery pseudoaneurysm and an aortic arch aneurysm. Composite graft replacement of the ascending aorta and aortic valve with reimplantation of the coronary arteries has some inherent complications. Our case report describes a patient with a pericomposite graft aneurysm presenting as a stenosis of the pulmonary artery, detected by Doppler echocardiography.
Adult↗
Primary balloon dilatation in coarctation of the aorta complicated by severe aortic insufficiency.
The combination of coarctation of the aorta in the presence of severe aortic insufficiency poses a serious clinical problem. Although successful single- and two-stage repair for combined coarctation in the presence of severe aortic regurgitation has been described, the surgical management of this lesion remains particularly difficult. The analysis of larger series of patients operated upon for coarctation reveals significant early mortality rate in patients with associated severe aortic insufficiency. Although the exact cause of the acute left ventricular failure remains unclear and is a matter of debate, one can assume that changes in the haemodynamics, resulting in global myocardial ischaemia from impaired coronary blood supply or a massive volume overload of the left ventricle after the correction of the coarctation, could have led to myocardial irritability and left ventricular failure. We present a three-stage repair with subtotal relief of the coarctation by balloon angioplasty and stenting first; elective aortic valve replacement in a second stage and finally total balloon dilatation of the residual stenosis at the previously subtotal dilated coarcted segment.
Adolescent↗
Chest pain in adult congenital heart disease: where does it come from?
Chest pain is common in adolescents and in young adults and usually not associated with a severe underlying cardiovascular disorder. However, in adults with congenital heart disease, residua or sequellae of previous interventions may provoke potential complications. Moreover, chest pain may be the first sign of a life-threatening condition. Basic knowledge is mandatory and will lead to the correct diagnosis and treatment. Data in literature, which focus on this issue, are scarce and motivated to summarize the experience of daily practice from the eye point of the clinician.
Adolescent↗
Ruptured aneurysm of the sinus of Valsalva into the right atrium. An uncommon congenital heart defect.
Rupture of an aneurysm of the sinus of Valsalva is an uncommon heart defect. A continuous murmur may be the first clinical sign of this rupture. Additional imaging techniques, and in the first place echocardiography, can be used to confirm the diagnosis. We present a case where, at the age 53 years, the diagnosis was made of a ruptured sinus of Valsalva into the right atrium, with a typical windsock image on echocardiogram. Although several cases of a ruptured sinus of Valsalva are reported in the literature, it is still an underdiagnosed condition because it is frequently missed during physical examination.
Aortic Aneurysm↗
Repair for partial and complete atrioventricular septal defect: single centre experience and long-term results.
BACKGROUND: The number of adult patients with surgical repaired congenital heart defects increases continuously. We were interested to compare late outcome after partial and complete atrioventricular defect repair (pAVSD and cAVSD, respectively) and to determine the most important reason for re-intervention. METHODS: All patients older than 16 years, who underwent partial or complete atrioventricular defect repair, were selected from the database. The medical files were reviewed for descriptive statistics. Kaplan Meier analysis was used to determine event free survival for both groups. Log rank testing was performed where applicable. RESULTS: One hundred thirty-eight patients were included. Two patients in each group with early post-operative mortality were excluded (pAVSD: 33/30 male/female, mean age 35.9 +/- 15.6 years; cAVSD: 27/ 44 male/female, mean age 25.7 +/- 11.9 years). Fourteen pAVSD-patients and 23 cAVSD-patients needed a surgical re-intervention: in two and 13 patients, respectively, a mitral valve repair was performed, and in five and one patient, respectively, a mechanical valve was replaced. Eight cAVSD-patients and 10 pAVSD-patients developed atrial arrhythmias. Seven patients (3 cAVSD and 4 pAVSD) received a pacemaker. Median event-free survival time was significantly shorter in the cAVSD-group (22.9 (95% CI 15.4-30.5) years) when compared to the pAVSD-group (34.0 (95% CI 20.1-47.9) years) (Log rank testing, P=0.017). CONCLUSION: Late outcome was characterized by a longer event free follow-up time of pAVSD-patients when compared to cAVSD-patients. The most important reason for re-intervention in both groups was mitral valve regurgitation. However, atrial arrhythmias and conduction disorders were not uncommon late after atrioventricular septal defect repair.
Adolescent↗