Humberto Morais

Thrombotic obstruction in left-side prosthetic valves: Role of thrombolytic therapy ☆

Prosthetic valve thrombosis (PVT) is a complication that endangers the life of patients with prosthetic heart valves. The incidence of PVT can be as high as 13% during the first year in any valve position and even 20% for mechanical prostheses in the tricuspid position. At any time, for prostheses in the mitral and/or aortic position, the overall incidence is 0.5–6% per patient-year, the highest in the mitral position. The risk of a thrombus, in spite of adequate oral anticoagulation, has been estimated at between 1% and 4% per year. The prevalence of asymptomatic nonobstructive PVT is 50%. During the early postoperative period, nonobstructive PVT may reach 10%.1 It is mandatory that the antithrombotic prophylaxis in these patients achieves an INR range of 2–4 as recommended by guidelines regardless of thrombogenic risk factors.2 PVT is classified as obstructive if there is a limitation of the leaflet mobility and a thrombus is present, clinically characterized by progressive heart failure, pulmonary edema or cardiogenic shock, and/or systemic embolism. In relation with auscultation, occluder clicks are typically muffled or absent. Also, stenotic or regurgitant murmurs may be heard. PVT is not obstructive when there is a thrombus but normal mobility of the leaflet, and this may not only cause stroke or peripheral embolism, but may also remain asymptomatic in about 50% of cases. Patient-related risk factors for PVT are: inadequate anticoagulation, left ventricular dysfunction, atrial fibrillation, pregnancy (hypercoagulability status), infection, early postoperative period, and spontaneous echocontrast as diagnosed by transoesophageal echocardiography (TEE).

Aneurysm of the right sinus of Valsalva dissecting into the interventricular septum, submitral aneurysm and left ventricular non-compaction: Three rare diseases in the same patient

A 24-year-old black man presented to the emergency department of our hospital with fatigue and dyspnea at rest of two weeks duration. The transthoracic echocardiogram (Figure 1) revealed aneurysm of the right sinus of Valsalva (Figure 1A) dissecting into the interventricular septum (arrow) (Figure 1B), dilated left ventricle with moderately to severely impaired systolic function, and echocardiographic criteria for left ventricular non-compaction (Figure 1C and D). A submitral aneurysm and a dilated left atrium were also observed (Figure 1C). Doppler echocardiography showed mild mitral and aortic regurgitation. Multislice computed tomography with contrast (Figure 2) revealed an aneurysm of the right sinus of Valsalva (Figure 2A) dissecting into the interventricular septum (arrow) (Figure 2B), as well as a submitral aneurysm (Figure 2C) and non-compaction of the left ventricle (Figure 2D), confirming the echocardiographic findings. The patient was admitted to the cardiac intensive care

Ventricular septal defect in children and adolescents in Angola: Experience of a tertiary center

INTRODUCTION AND OBJECTIVE This is the first study in Angola with the aim of characterizing ventricular septal defect (VSD) among children and adolescents. METHODS A cross-sectional study based on echocardiographic records of the largest pediatric cardiology center in Angola included all children and adolescents (0 to 18 years old) with VSD between April 2010 and March 2011. The diagnosis was made by transthoracic and Doppler echocardiography with a Medison SA 8000 system. The sample was divided into two groups: Group 1, isolated VSD; and Group 2, VSD associated with other congenital heart defects (CHDs). Age, gender, type of VSD, associated CHDs and genetic syndromes were assessed. RESULTS A total of 490 CHDs were diagnosed, of which 283 were VSDs. In Group 1 (140, 49%) the mean age was 29±36 months. The most frequent age (mode) at diagnosis was 24 months. There was no predominance of gender (ratio 1:1). The majority (127, 91%) had perimembranous VSD. In Group 2 (143, 51%) 113 patients (79%) had one, 27 patients (19%) had two and three patients (2%) had three other CHDs. Trisomy 21 was the most common genetic syndrome (23, 96%). CONCLUSIONS The study shows that VSD is the most common CHD in childhood, the diagnosis is made late and almost half of VSDs are associated with other CHDs.

A Right Upper Lobe Mass in the Thorax After Modified Blalock-Taussig Shunt.

We report an incidental finding of pseudoaneurysm in a 10-month-old boy with tetralogy of Fallot and Down syndrome who had undergone placement of a modified Blalock-Taussig shunt at age four months. Computed tomography was a determinant exam for better assessment. The lesion was successfully resected with concomitant complete repair of tetralogy of Fallot in a single-stage. The child was asymptomatic at fourth month follow-up.

Submitral aneurysm and the new imaging modalities: Will magnetic resonance imaging be necessary?

Submitral aneurysm is a rare cardiac malformation commonly reported in young adult African ancestry. Transthoracic echocardiogram is a gold standard test for diagnosis. Magnetic resonance imaging provides detailed anatomic and functional information of the heart. We present a case of a large bilobed submitral aneurysm in-witch the magnetic resonance imaging confirmed the same findings of the transthoracic echocardiography and in addiction also showed a parietal thrombus.

Anomalous Origin of One Pulmonary Artery From the Ascending Aorta From Diagnosis to Treatment in Angola

Background: Anomalous origin of one pulmonary artery is a rare congenital heart disease in which one pulmonary artery branch originates from the ascending aorta. Objective: To describe the experience of a cardiothoracic center in an African country to repair anomalous origin of one pulmonary artery in the context of Portugal–Angola collaboration. Methods: Between March 2011 and March 2015, four consecutive patients with anomalous origin of pulmonary artery branch underwent surgical correction. The mean age was 1.6 months. The mean weight was 4 kg. All had right pulmonary artery branch originating from the ascending aorta. All patients underwent direct implantation of right pulmonary branch to main pulmonary artery. Two patents had patent ductus arteriosus and one had atrial septal defect. Two patients had pulmonary hypertension. Results: There was no registration of death. The mean cardiopulmonary bypass time was 75.5 ± 4.5 minutes, mean aortic cross-clamping time was 40 ± 5.6 minutes, and mean duration of the postoperative intensive care unit stay was 6.8 ± 5.7 days. At discharge, one patient had residual gradient of 25 mm Hg, the remainder had no significant gradient. The mean follow-up time was 11 months (5-28 months). One week after discharge, one patient presented operative wound dehiscence. At the last follow-up, all patients were alive, and no significant residual gradient or stenosis at site of anastomosis was observed. No reintervention was required. Conclusion: Anomalous origin of one pulmonary artery is a rare but potentially treatable lesion if operated early in life. Direct implantation was a good technique with good short-term results.

Ebstein's anomaly in children: A single-center study in Angola

INTRODUCTION AND OBJECTIVE Ebsteins anomaly is a rare complex congenital heart defect of the tricuspid valve. We aimed to describe the frequency, clinical profile, and early and short-term post-operative results in patients under the age of 18 years operated for this anomaly in a tertiary center in Angola. METHODS A retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects. RESULTS Of the 1362 patients studied, eight (0.6%) had Ebsteins anomaly; six patients (75%) were female. Mean age was 69±59 months. Five patients were in NYHA functional class III or IV. Mean cardiothoracic index was 0.72. Seven patients (87.5%) had severe tricuspid regurgitation and five (62.5%) had another associated congenital heart defect. All patients were operated: two had complications and one (12.5%) died in the early post-operative period. The mean follow-up time was 1.22±0.6 years, and mortality during follow-up was 12.5% (n=1). At the end of the study, of the five patients in whom cone reconstruction was performed, four (80%) were in functional class I. Mean cardiothoracic index decreased to 0.64. Three patients had mild and two had moderate tricuspid regurgitation. The patient who underwent cone reconstruction and a Glenn procedure was in functional class I. CONCLUSION The frequency of Ebsteins anomaly was similar to that in other centers. Cone reconstruction was viable in the majority of patients, with good early and short-term results.

diagnostic assessment of prosthetic mitral valve thrombosis by real-time three-dimensional transoesophageal echocardiography and successful thrombolytic treatment

Prosthetic valve thrombosis (PVT) is a rare but serious complication of valve replacement, most often encountered with mechanical prostheses. The different therapeutic modalities for PVT (fibrinolysis with heparin treatment or surgery) will largely be influenced by the presence of valvular obstruction, the valve location (left or right sided), the patients clinical status, the existence of and expertise in therapeutic modalities at the institution, and the patients decision. This report describes a patient with thrombosis of a prosthetic mitral valve, which was successfully treated with recombinant streptokinase in a hospital without cardiac surgery. In this context, the authors present the real-time transoesophageal echocardiographic appearance of this complication, and give a brief review of the literature.

Multimodality imaging of a huge subaortic left ventricular aneurysm in a child

A subannular left ventricular aneurysm is very rare, and is mostly considered to be a congenital anomaly. A subannular left ventricular aneurysm is classified based on the type of its own orifice—submitral or subaortic. Subaortic left ventricular aneurysm occurs less frequently compared with a submitral type of subannular aneurysm. We hereby describe a rare case of a huge bilobed subaortic aneurysm, in which the orifice was located just below the left coronary cusp diagnosed with multimodality imaging in a child.

Rupture of Right Sinus of Valsalva Aneurysm into Right Cardiac Chambers: The Role of Different Imaging Modalities

A 25-year-old woman with no cardiovascular risk factors was admitted to our hospital with dyspnea and palpitations during the last two months. Physical examination showed blood pressure of 150/50 mm Hg and a continuous murmur across the precordium in cardiac auscultation was observed. The 12 leads electrocardiogram was normal. Chest X-ray showed a slight cardiomegaly. The transthoracic echocardiography revealed dilatation of the right cavities and a rupture of right sinus of Valsalva aneurysm (SVA) into the right atrium (Fig. 1A). The Doppler study (Fig. 1B) and transesophageal echocardiography confirmed the shunt between the right SVA and the right atrium (Fig. 1C and D, Supplementary movie 1 and 2). The rupture of right sinus of Valsalva was also demonstrated by real time 3D transesophageal echocardiography (Fig. 2A, Supplementary movie 3). Unlike transthoracic and transesophageal echocardiography which shows shunt between right SVA and right atrium, computed multidetector tomography, showed a communication between the right SVA and right cardiac chambers (right atrium and right ventricle) (blue circle, Fig. 2B). These findings were confirmed during surgery (blue arrow, Fig. 2C), and the fistula was closed (Fig. 2D). The patients postoperative course was uneventful. The patient was discharged on the sixth post-operative day. At two years follow-up, the patient remains asymptomatic. Fig. 1 A: Transthoracic echocardiography, parasternal short axis view at level of great vessels, revealed rupture of right sinus of Valsalva into the right atrium (asterisk). B, C, and D: Transesophageal echocardiography and color Doppler study confirming the ... Fig. 2 A: Real time 3D transesophageal echocardiography, in zoom mode, showing rupture of right sinus of Valsalva into the right atrium (blue arrow). B: Computed multidetector tomography showing communication between the right sinus of Valsalva and right cardiac ... SVA are rare but well known congenital anomalies. They occur three times more often in males with highest incidence in Asian populations. They commonly rupture into the right ventricle or right atrium,1) rarely into the both right cardiac chambers as was observed in case presented herein. Moreover, in Africa the main complication of the SVA is the dissection into ventricular septum.2) Since the first echocardiographic description of SVA by Rothbaum et al.3) in 1974, the echocardiography plays a pivotal role in the definitive diagnosis aneurysm of the sinus of Valsalva. However, in recent years several papers have been published emphasizing the use of other imaging modalities for diagnosis of this heart condition.4),5) According to several reports, the diagnosis of SVA was made at echocardiography in 90% of cases. In present case the multidetector tomography gave more precise information showing a rare type of rupture of SVA-rupture into right cardiac chambers, and the role of multidetector tomography as additional tool for the precision of the diagnosis. In present case the detailed anatomic and functional information obtained by these non-invasive tests were enough to guide the surgical planning without recourse to invasive studies. Invasive tests must be reserved for patients in whom the percutaneous closure of the fistula is possible, or the suspicion of coronary disease is present. Finally, given the widespread use of these new noninvasive tests, cardiologists and radiologists should be able to recognize this rare disease.