scholarly journals P1337 Left ventricle outpouchings. Assesing diagnostic complexity

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Blasco Turrion ◽  
L Marcos Fuentes ◽  
A Moruno Tirado ◽  
F Coserria Sanchez ◽  
I Valverde Perez

Abstract Congenital left ventricle outpouchings (LVO) are a very rare condition that include left ventricle accessory chamber, left ventricle aneurysm (LVA), left ventricle diverticulum (LVD), double-chambered left ventricle and accessory left ventricle, without well-stablished diagnostic criteria. A recent article by Elaheh Malakan et al tries to show this difficulties and propose an algorithm to help define this different pathologies. We present the case of a 26-day-old boy that was admitted to our Hospital with suspicion of dilated cardiomyopathy. In his physical exam a systolic murmur was detected, and his mother described tiredness during intakes and tachypnea. His ECG showed a sinus rhythm, with an 80º QRS axis. No signs of hypertrophy or dilated cavities were detected. As part of the study a TTE was performed, detecting a dilated left ventricle with a tract crossing the apical side from the LV lateral wall. The MRI confirmed the presence of an incomplete muscular septum from de LV lateral wall up to the apex encompassing the papillary muscles in the mid left ventricle, dividing the LV in two chambers with telediastolic size of 3.5x3cm and 2.6x2.4cm respectively. This secondary appendix, with a pseudoaneurysm morphology, distended in systole with a dyskinetic motion. Globally, the left ventricle was dilated and had mild systolic disfunction. The right cavities were morphological and functionally normal. In this case initially a common dilated cardiomyopathy was suspected, being able to confirm the presence of a muscular tract crossing the mid left ventricle. The MRI described the image as an LV pseudoaneurysm, however the TTE images and the diagnostic algorithm proposed by Elaheh Malakan et al would include this case as a two-chamber left ventricle, denoting the still present difficulties in the diagnostic process in these cases, and its impact in their therapeutic approach. Abstract P1337 Figure.

2020 ◽  
Vol 23 (5) ◽  
pp. E586-E589
Author(s):  
Seung Hun Lee ◽  
Jae Young Cho ◽  
Ho Sung Sho ◽  
Cheol Woong Yu

Background: Coronary artery fistula (CAF) draining into the left ventricle (LV) is a rare condition and dilated cardiomyopathy (DCMP) that results from single coronary artery (SCA) accompanied by CAF also is extremely rare. Case report: We report the case of a 36-year-old man, who presented with severe DCMP that resulted from SCA with CAF draining into the LV. Transthoracic echocardiogram (TTE) showed severe diffuse hypokinesia of the LV with ejection fraction (EF) of 15-20%. Coronary angiography (CAG) revealed SCA connected between left anterior descending artery (LAD) and posterior descending artery (PDA), course of the PDA was very tortuous from apex to base of the LV, and connected to posterior lateral (PL) branch, which was drained into the LV at distal part of the PL. Coronary artery computed tomography (CACT) showed LAD ran over the apex of the LV and connected to PDA, which was drained into the mid portion of lateral wall of the LV. Cardiac magnetic resonance imaging (CMRI) showed no evidence of irreversible myocardial change in global wall of the LV. The patient underwent surgical ligation of PDA near the base of the posterior wall of the LV as close to the entry of CAF to the LV as possible without any surgery-related complications. Three months after the surgical ligation, follow-up TEE  showed much improved EF of 45-50%. He has been doing well without congestive heart failure (CHF) until now. Discussion: Symptomatic CAF with hemodynamic deterioration may need mechanical correction of CAF, including surgical ligation or percutaneous interventional occlusion. How to treat this condition in terms of methodology is a very difficult issue. The detailed methods related to surgical or interventional correction of CAF have to be determined based on anatomical characteristics of CAF, underlying comorbidities, and relevant complications risk.


2014 ◽  
Vol 31 (04) ◽  
pp. 225-227
Author(s):  
R. Miguel ◽  
R. Reinaldo ◽  
K. Fraga ◽  
A. Galvão ◽  
J. Silva ◽  
...  

Abstract Introduction: Caracara plancus, popularly known as Carcará, is a bird with broad geographic distribution, occurring from the southern United States to Argentina. Owing to importance of the heart during the flight and the lack of studies of this body in caracaras, this research aimed to conduct the description of the morphology and internal and external morphology heart Caracara plancus. Materials and Methods: Using a magnifying glass (TECNIVAL, SQF-F), precision scale and steel caliper, the heart three caracaras were described morphologically, weighed and measured. Results: The weight of hearts averaged 7,246g ± 0,518g. The mean side-to-side axis was 2,196cm ± 0,085cm and 3,366cm ± 0,036cm the longitudinal axis. In the side wall of the right ventricle was obtained the value of 0,185cm ± 0,035cm in the lateral wall of the left ventricle 0,59cm ± 0,014cm and interventricular septum 0,014cm ± 0.52cm. The heart of Carcará has no anterior interventricular groove; however there is the presence of the posterior interventricular sulcus. Internally, the left ventricle was observed, the presence of atrioventricular valves formed by connective tissue 18 tendinous cords, papillary muscles and a bridge crests. In the right ventricle, the atrioventricular valve was formed by muscle tissue and did not submit chordae, papillary muscles, crests or bridge. Conclusion: The heart of Carcará features in its morphology similar to the hearts of other birds and can be checked one right atrioventricular valve constituted by muscle tissue.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Blasco Turrion ◽  
I Guillen Rodriguez ◽  
A Moruno Tirado ◽  
A Mendez ◽  
I Valverde Perez

Abstract Dilated cardiomyopathy (DCM) is defined as left ventricle (LV) dilatation and dysfunction in the absence of abnormal loading conditions or coronary artery disease enough to cause global systolic impairment. It is the leading cause of heart failure and sudden cardiac death, and even in the early stages of life 5,7/1.000.000 children are diagnosed each year. Lately, genetic screening has revealed that 30-50% of the cases have a familial origin, with a high genetic heterogeneity. We present the case of a 4-month-old boy (case A) referred to our Hospital with high suspicion of dilated cardiomyopathy. He had a twin brother, pregnancy went without complications, pre-birth ultrasound and blood tests were completely normal and a caesarean birth was planned at 37 weeks of pregnancy. He also has an older sibling with no medical history. Our patient was the first to be born and was admitted to the neonatal-ICU due to tachypnea, labored breathing and desaturation. In the physical exam a 5cm hepatomegaly was detected and on the X-Ray a cardiomegaly and enlarged mediastinum were confirmed. ECG showed no electrical abnormalities besides vague repolarization changes. A TTE was done, showing remarkable left cavities enlargement with severe left ventricle dysfunction and moderate mitral regurgitation, comprising the right filling and therefore the right cardiac output. These findings were confirmed by MRI, not detecting enhancement patterns compatible with myocarditis. A genetic test was performed in the index case detecting the presence of a mutation in the desmin gene (Des C.568 + 10c) and the myosin heavy chain 7 gene (Myh7p - glu883Ala) both associated either with dilated and noncompaction cardyomiopathies. Due to the elevated chances of a familiar-DCM his twin brother was admitted to study (case B), with no symptoms or clinical signs besides an isolated bronchitis episode. The clinical examination and EKG were normal, detecting in the TTE a slightly dilated left ventricle with non-compactation morphology in the LV lateral wall, but no left ventricle dysfunction. Also, a TTE was performed in their father with similar findings: mild LV dilatation with minor systolic disfunction and uncertain non-compactation morphology in the LVl apex. Abnormalities of specific genes are now known to be responsible for different types of congenital heart diseases with important implications in genetic counseling. We want to emphasize the different manifestation of a single mutation on different members of a family, even in this two twin-brothers that, under the same probable conditions during preganancy, had slightly different cardyomyopathies but very different severity, and probable mid to long term prognosis. Abstract 1106 Figure.


2021 ◽  
Vol 11 (20) ◽  
pp. 9427
Author(s):  
Mihaela Camelia Tîrnovanu ◽  
Vlad Gabriel Tîrnovanu ◽  
Bogdan Florin Toma ◽  
Elena Cojocaru ◽  
Carmen Ungureanu ◽  
...  

Struma ovarii is a rare condition with scarce published data regarding clinical, morphological, and therapeutic approaches. This study reports the experience of 25 patients with struma ovarii who received surgical treatment in a gynecology department in Romania. The study was conducted from January 1999 to September 2021 and included patients with confirmed struma ovarii whose medical records were retrospectively reviewed and evaluated. Struma ovarii represented 2.8% of the total number of benign ovarian tumors treated by surgery. The age of the patients was between 24 and 71 years. The tumor was unilateral in 24 cases, 13 cases on the left ovary, 11 on the right side, and bilateral in 1 case. Tumor dimensions ranged between 1 cm and 20 cm. In two cases, the patients had symptoms of hyperthyroidism. The procedure was performed on four women for diagnoses other than an ovarian tumor. In another five situations, there was suspicion of ovarian malignancy. In addition, struma ovarii was associated with other clinical conditions in 22 cases. These lesions represent a diagnostic challenge with heterogeneous clinical and imaging manifestations. Complete information of clinical, morphologic, and surgical findings may improve the diagnostic algorithm and better predict patient outcomes.


2008 ◽  
Vol 53 (No. 3) ◽  
pp. 165-168 ◽  
Author(s):  
W. Perez ◽  
M. Lima ◽  
G. Pedrana ◽  
F. Cirillo

In the present study the most outstanding anatomical findings of the heart of a giraffe are described. Two papillary muscles were found in the right ventricle, namely magnus and subarterial. There were no papillary parvi muscles. The supraventricular crest gave insertion to various tendinous chords. These chords fixed the angular cusp of the right atrioventricular valve. The pectinate muscles were better developed in the left auricle than in the right one. Within the left ventricle two big papillary muscles were found as well as a notorious septomarginal trabecula. The left coronary artery irrigated the majority of the heart’s territory. It gave origin to the interventricular paraconal branch and to the circumflex branch. The latter gave off the branch of the left ventricular border and the interventricular subsinosal branch.


1991 ◽  
Vol 1 (2) ◽  
pp. 152-154 ◽  
Author(s):  
Jeong-Wook Seo ◽  
Woo Hee Jung ◽  
Yong Won Park

SummaryAnatomic data are presented of a female abortus with the unusual combination of Ebstein's malformation and atrioventricular septal defect with right isomerism of the atrial appendages, left-hand ventricular topology and discordant ventriculoarterial connection. The common atrioventricular orifice was guarded by an effectively common valve, the right half being guarded by a well-formed three-leaflet valve, but the posterior and left lateral wall of the orifice being deficient of valvar leaflet tissue. The right ventricular inlet was a blind chamber, an imperforate dimple being seen which was surrounded by valvar remnants. The apical trabecular and outlet components of the right ventricle communicated with the left ventricle through an outlet ventricular septal defect in front of the anterosuperior leaflet of the atrioventricular valve, giving the potential clinically for the lesion to be misdiagnosed as double inlet left ventricle.


2008 ◽  
pp. 507-515
Author(s):  
J Švíglerová ◽  
J Kuncová ◽  
L Nalos ◽  
J Slavíková ◽  
M Štengl

The contribution of the sympathetic innervation to the postnatal development of cardiac contractility remains unclear. In this study, the postnatal maturation of cardiac contractility was compared in control rats and rats after chemical sympathectomy. The chemical sympathectomy was induced by administration of 6-hydroxydopamine to newborn rats. At days 20, 40 and 60 of postnatal life, the contractile parameters and concentrations of sympathetic neurotransmitters were measured in both right and left ventricles. In rats with chemical sympathectomy, concentrations of norepinephrine were reduced almost completely in both ventricles at all time points. The contractility of the left ventricle papillary muscles was substantially decreased at all time points. In contrast, the contractility of the right ventricle papillary muscles was decreased only transiently, showing a recovery at day 60 regardless of the permanently decreased concentration of norepinephrine. The concentration of neuropeptide Y, another neurotransmitter present in sympathetic nerves, showed the same developmental trend as contractility: permanent reduction in the left ventricle, transient reduction with a recovery at day 60 in the right ventricle. The data indicate that the sympathetic nervous system plays an important role in the postnatal development of cardiac contractility and neuropeptide Y may contribute to this effect.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S81-S81
Author(s):  
D Ibrahim ◽  
A Gohara

Abstract Casestudy: Hypertrophic cardiomyopathy is a heterogeneous disease with a number of phenotypes. We present the rarest phenotype, 1% of all cases, the midventricular obstructive type. It is characterized by hypertrophy in the mid portion of the left ventricle involving the papillary muscles, resulting in systolic intraluminal obstruction of the mid-ventricle. An unusual case of a 41-year-old male who died suddenly while hiking. He had no past medical history except for hyperlipidemia. He had no history of hypertension, with documented repeated normal blood pressures. No family history of cardiac problems, sudden death, or death at young age. On autopsy examination, his heart weighed 540 grams. The left ventricle was concentrically hypertrophied, 1.8 cm in thickness, with prominent papillary muscles and small lumen. The interventricular septum measured 1.8 cm in thickness, with increase sub-aortic wall thickness. Microscopic examination revealed myocyte hypertrophy with boxcar-like nuclear enlargement and interstitial fibrosis. There was myocardial fiber disarray. Intramural vessels showed dysplastic changes with medial thickening and narrowing of the lumen. Genetic cardiomyopathy panel testing on paraffin-embedded sections was negative; testing-on whole blood is preferred but body was embalmed before autopsy. The immediate cause of death was hypertrophic cardiomyopathy leading to pulmonary edema with potential complications as arrhythmias and low cardiac output. This case highlights the importance of light microscopy in the diagnostic process despite negative genetics. It also underscores the crucially of adopting better strategies to identify the disease since sudden death occurs frequently in those where diagnosis is missed during life.


1988 ◽  
Vol 255 (6) ◽  
pp. H1370-H1377 ◽  
Author(s):  
B. D. Hoit ◽  
W. Y. Lew ◽  
M. LeWinter

We studied eight open-chest dogs to determine whether there is regional variation in pericardial contact pressure (PCP). Flat, air-filled balloons were used to measure PCP simultaneously over the lateral walls of the right and left ventricles while cardiac volume was varied by dextran infusion. End-diastolic and mean PCP were significantly higher over the left than right ventricle at high (20.3 +/- 1.0 mmHg) and middle levels (13.7 +/- 0.9 mmHg) of left atrial pressure. At high left atrial pressures, the end-diastolic PCP over the lateral left ventricle was 9.1 +/- 2.4 mmHg compared with 4.3 +/- 2.3 mmHg over the lateral right ventricle (P less than 0.05). At middle levels of left atrial pressures, end-diastolic PCP was 6.2 +/- 3.5 mmHg over the left ventricle and 1.5 +/- 2.4 mmHg over the right ventricle (P less than 0.05). These variations in PCP persisted after severing the pericardial diaphragmatic attachments and after turning the dogs such that one or the other balloon was dependent. Regional distribution of PCP was studied by positioning a single balloon sequentially at multiple ventricular sites. PCP was consistently higher over the lateral wall of the left ventricle than either the anterior or posterior walls of the right or left ventricle. After aortic occlusion, end-diastolic PCP increased more over the left than right ventricle. In contrast, with pulmonary artery occlusion, end-diastolic PCP increased more over the right than left ventricle. Pericardial pressure varies regionally, and a single pericardial pressure may be an oversimplification when used to describe pericardial restraint on the cardiac volume.


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