scholarly journals A misinterpretation of the left ventricular non-compactionadult patient with primary pulmonary hypertension

2011 ◽  
Vol 64 (11-12) ◽  
pp. 597-599 ◽  
Author(s):  
Aleksandra Nikolic ◽  
Ljiljana Jovovic ◽  
Velibor Ristic ◽  
Dejan Nikolic ◽  
Lazar Angelkov

Non-compaction of the left ventricle is a rare cardiac malformation, defined as a primary cardiomyopathy caused by genetic malformations. Although the pathogenesis of this cardiomyopathy is unknown, there are two possible hypotheses (congenital and acquired) which lead to arrest in intrauterine endomyocardial morphogenesis. We are presenting a case of a 60-year-old woman, with a history of bradyarrhythmia, syncope and cyanosis. Two-dimensional echocardiography showed the thickened myocardium with prominent trabeculations and deep intertrabecular recesses in the two thirds of the apical part of left ventricle walls. The right side cavity was enlarged with hypertrophied wall. Tricuspid regurgitation was moderate. Systolic pressure in the right ventricle was 70mmHg. Catheterization of the right heart showed high pressure in the pulmonary artery. According to publications, this is a very rare case with the presence of possible primary pulmonary hypertension and non-compaction of the left ventricle.

1987 ◽  
Vol 253 (6) ◽  
pp. H1381-H1390 ◽  
Author(s):  
W. L. Maughan ◽  
K. Sunagawa ◽  
K. Sagawa

To analyze the interaction between the right and left ventricle, we developed a model that consists of three functional elastic compartments (left ventricular free wall, septal, and right ventricular free wall compartments). Using 10 isolated blood-perfused canine hearts, we determined the end-systolic volume elastance of each of these three compartments. The functional septum was by far stiffer for either direction [47.2 +/- 7.2 (SE) mmHg/ml when pushed from left ventricle and 44.6 +/- 6.8 when pushed from right ventricle] than ventricular free walls [6.8 +/- 0.9 mmHg/ml for left ventricle and 2.9 +/- 0.2 for right ventricle]. The model prediction that right-to-left ventricular interaction (GRL) would be about twice as large as left-to-right interaction (GLR) was tested by direct measurement of changes in isovolumic peak pressure in one ventricle while the systolic pressure of the contralateral ventricle was varied. GRL thus measured was about twice GLR (0.146 +/- 0.003 vs. 0.08 +/- 0.001). In a separate protocol the end-systolic pressure-volume relationship (ESPVR) of each ventricle was measured while the contralateral ventricle was alternatively empty and while systolic pressure was maintained at a fixed value. The cross-talk gain was derived by dividing the amount of upward shift of the ESPVR by the systolic pressure difference in the other ventricle. Again GRL measured about twice GLR (0.126 +/- 0.002 vs. 0.065 +/- 0.008). There was no statistical difference between the gains determined by each of the three methods (predicted from the compartment elastances, measured directly, or calculated from shifts in the ESPVR). We conclude that systolic cross-talk gain was twice as large from right to left as from left to right and that the three-compartment volume elastance model is a powerful concept in interpreting ventricular cross talk.


2015 ◽  
Vol 156 (28) ◽  
pp. 1140-1143
Author(s):  
István Hartyánszky ◽  
Márta Katona ◽  
Krisztina Kádár ◽  
Asztrid Apor ◽  
Sándor Varga ◽  
...  

Aortico-left ventricular tunnel is a rare congenital cardiac defect, which bypasses the aortic valve via the paravalvar connection from the aorta to the left ventricle. The authors report the history of a 14-year-old boy with aortico-left ventricular tunnel in whom the aortic orifice arose from the right aortic sinus and was closed by a pericardial patch. The diagnosis was confirmed by combined two-dimensional and real time three-dimensional echocardiogram and magnetic resonance imaging. This is the first case, in which these complex diagnostic imaging methods have been used in the pre- and postoperative management of this defect. Optimally the new transthoratic three-dimensional echocardiography would be needed to define the anatomy and functional consequences of the aortico-left ventricular tunnel and in the postoperative follow-up. Orv. Hetil., 2015, 156(28), 1140–1143.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2950-2950
Author(s):  
John Allen Steuter ◽  
Philip Bierman ◽  
R. Gregory Bociek ◽  
Martin Bast

Abstract Anthracycline-based chemotherapy is widely used in a variety of regimens for Hodgkin’s Lymphoma (HL) and Non-Hodgkin’s Lymphoma (NHL). Unfortunately this agent is associated with cardiotoxicity, especially in larger cumulative doses. Doxorubicin cardiotoxicity is characterized by a dose-dependent decline in mitochondrial oxidative phosphorylation. Reactive oxygen species, generated by the interaction of doxorubicin with iron, can then damage the myocytes causing myofibrillar loss, cytoplasmic vacuolization, and apoptosis. Current NCCN guidelines for HL and NHL state that left ventricle function is recommended in patients who will receive such chemotherapy. Subsequent evaluations are recommended at a cumulative dose of doxorubicin of at least 300 mg/m² and periodically thereafter during the course of therapy. Despite these recommendations, there is little literature about the usefulness of this approach, the rate of abnormal findings, and whether routine testing changes management. A list of patients with HL or NHL treated with anthracycline-based chemotherapy from the University of Nebraska Medical Center from August 2004-May 2012 was obtained from our Lymphoma Registry. Baseline characteristics of age, disease diagnosis, gender, and prior history of cardiac diagnosis were collected. Prior cardiac diagnosis specified as patients with a past history of atrial fibrillation/flutter, supraventricular tachycardia, heart block requiring pacemaker implantation, coronary artery disease, valvular disease, pulmonary hypertension, or cardiomyopathy. Charts were reviewed for pre-chemotherapy evaluation of left ventricular function and the method of evaluation (echocardiogram, nuclear, cardiac MRI were included). Additionally, post treatment evaluation of cardiac function was evaluated for any change. The individual therapy for each patient was reviewed to determine if findings from the cardiac evaluation modified treatment regimens. A left ventricular function less than 50% was considered abnormal. Echocardiogram findings of moderate to severe valvular disease, diastolic dysfunction (grade 1-3), and moderate or severe pulmonary hypertension (moderate = pulmonary artery systolic pressure of 45-60, severe = pulmonary artery systolic pressure of > 60) were collected. We identified 309 patients from the UNMC lymphoma database. Of these 219 (71%) had an echocardiogram performed prior to therapy and documented in the patient record. Their mean age was 54.2 years of age with 53% of them being male and 47% female. 188 of the 219 (86%) had no prior cardiac diagnosis as defined previously. From this group 22 of the 188 (10%) had a pre-chemotherapy echocardiogram that demonstrated one of the following: moderate-severe valvular disease, diastolic dysfunction, moderate-severe pulmonary hypertension or abnormal EF (<50%). However, none of these findings altered the chemotherapy regimen for the 22 patients. 31 of the 219 patients with echocardiograms carried a prior cardiac diagnosis. 4 of the 31 had an alteration in their chemotherapy regimen as a result. Post therapy echocardiograms showed no change in cardiac function. 5 of the 219 patients were diagnosed with adriamycin induced cardiomyopathy following treatment. All 5 patients had no prior cardiac history and pre-chemotherapy echocardiograms were normal. These finding suggest that current methods of evaluating cardiac function prior to chemotherapy and risk stratifying patients are inadequate and do not alter patient outcomes. Disclosures: No relevant conflicts of interest to declare.


1991 ◽  
Vol 261 (1) ◽  
pp. H212-H219 ◽  
Author(s):  
J. M. Capasso ◽  
P. Li ◽  
G. Guideri ◽  
P. Anversa

To determine whether moderate ingestion of alcohol for protracted periods of time affects normal cardiac performance and produces myocyte damage, male Fischer 344 rats at 4 mo of age were given 30% ethanol in their drinking water every day for a period of 8 mo. Experimental animals and age-matched controls were examined hemodynamically and morphometrically at 12 mo of age. Body and cardiac growth were depressed in alcoholic animals by 15 and 12%, respectively. Although left ventricular (LV) weight was reduced by 14% in alcoholic rats, no difference in right ventricular (RV) weight was noted, and consequently the ratio of RV weight to body weight increased by 12%. Systemic arterial pressures as well as LV peak systolic pressure decreased in alcoholic rats despite an unchanged heart rate. Myocardial contractility in alcoholic rats was further depressed as revealed by a significant decrease in the peak rate of ventricular pressure decay. Importantly, end-diastolic pressure was elevated 5.2-fold in the left ventricle and 2.9-fold in the right ventricle after 8 mo of ethanol consumption. LV diastolic chamber volume increased through myocardial remodeling as the longitudinal axis and transverse diameters from the base to the apex increased in experimental animals while the thickness of the LV diminished. Structural and hemodynamic alterations resulted in a 571% increase in the volume of diastolic circumferential wall stress on the left ventricle. Damage to the myocardium was increased in alcoholic animals with the volume percent of myocardial lesions increasing 342% in the wall of the left ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)


2018 ◽  
Vol 9 (1) ◽  
pp. 204589401881697
Author(s):  
Enric Domingo ◽  
Juan C. Grignola ◽  
Pedro Trujillo ◽  
Rio Aguilar ◽  
Antonio Roman

Regression of pulmonary hypertension (PH) is often incomplete after successful left-sided valve replacement (LSVR). Proximal pulmonary arterial (PPA) wall disease can be involved in patients with persistent-PH after LSVR, affecting the right ventricular to pulmonary arterial (RV-PA) coupling. Fifteen patients underwent successful LSVR at least one year ago presenting PH by echo (> 50 mmHg). Prosthesis-patient mismatch and left ventricular dysfunction were discarded. All patients underwent hemodynamic and intravascular ultrasound (IVUS) study. We estimated PPA stiffness (elastic modulus [EM]) and the relative area wall thickness (AWT). Acute vasoreactivity was assessed by inhaled nitric oxide (iNO) testing. RV-PA coupling was estimated by the tricuspid annular plane systolic excursion to systolic pulmonary arterial pressure ratio. Patients were classified as isolated post-capillary PH (Ipc-PH; pulmonary vascular resistance [PVR] ≤ 3 WU and/or diastolic pulmonary gradient [DPG] < 7 mmHg) and combined post- and pre-capillary PH (Cpc-PH; PVR > 3 WU and DPG ≥ 7 mmHg). Both Ipc-PH and Cpc-PH showed a significant increase of EM and AWT. Despite normal PVR and DPG, Ipc-PH had a significant decrease in pulmonary arterial capacitance and RV-PA coupling impairment. Cpc-PH had worse PA stiffness and RV-PA coupling to Ipc-PH ( P < 0.05). iNO decreased RV afterload, improving the cardiac index and stroke volume only in Cpc-PH ( P < 0.05). Patients with persistent PH after successful LSVR have PPA wall disease and RV-PA coupling impairment beyond the hemodynamic phenotype. Cpc-PH is responsive to iNO, having the worse PA stiffness and RV-PA coupling. The PPA remodeling could be an early event in the natural history of PH associated with left heart disease.


2018 ◽  
Vol 5 (3) ◽  
pp. K59-K62 ◽  
Author(s):  
Sothinathan Gurunathan ◽  
Roxy Senior

Summary We present the case of a 32-year-old man who presented with a remote history of chest pain and was diagnosed with non-compaction cardiomyopathy on echocardiography. On presentation, he was relatively asymptomatic with normal cardiac function. Unfortunately, he presented 1 year later with a catastrophic embolic stroke. Learning points: Left ventricular non-compaction (LVNC) is a myocardial disorder characterised by prominent left ventricular (LV) trabeculae, a thin compacted layer and deep intertrabecular recesses. Two-dimensional echocardiography with colour Doppler is the study of choice for diagnosis and follow-up of LVNC. CMR serves an important role where adequate echocardiographic imaging cannot be obtained. LVNC is associated with high rates of mortality and morbidity in adults, including heart failure, thromboembolic events and tachyarrhythmias.


Author(s):  
Francesca R Pluchinotta ◽  
Vivek Muthurangu

Supravalvar aortic stenosis, aortic arch anomalies, and coarctation of the aorta are lesions involving the ascending and descending aortas, which can cause obstruction to left ventricular forward flow. In some cases, they can be associated with an anomalous borderline left ventricle. Obstructive lesions impose an increased afterload on the left ventricle and, if severe and untreated, result in hypertrophy and eventual dilatation and failure of the left ventricle. In addition, clinical manifestations of double aortic arches and other vascular rings include swallowing or respiratory symptoms due to extrinsic compression. In most cases, the anatomical diagnosis can be made from two-dimensional echocardiography. Common indications for performing cardiovascular magnetic resonance (CMR) are to refine an echocardiographic diagnosis before selecting a management plan or during follow-up to depict post-operative changes after surgical correction or catheter intervention. The goals of CMR in the assessment of patients with left-sided heart lesions are anatomical evaluation of the location, functional assessment of the haemodynamic burden, and identification of associated anomalies.


2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Pratik K. Dalal ◽  
Amy Mertens ◽  
Dinesh Shah ◽  
Ivan Hanson

Acute myocardial infarction (AMI) resulting in cardiogenic shock continues to be a substantial source of morbidity and mortality despite advances in recognition and treatment. Prior to the advent of percutaneous and more durable left ventricular support devices, prompt revascularization with the addition of vasopressors and inotropes were the standard of care in the management of this critical population. Recent published studies have shown that in addition to prompt revascularization, unloading of the left ventricle with the placement of the Impella percutaneous axillary flow pump can lead to improvement in mortality. Parameters such as the cardiac power output (CPO) and pulmonary artery pulsatility index (PAPi), obtained through pulmonary artery catheterization, can help ascertain the productivity of right and left ventricular function. Utilization of these parameters can provide the information necessary to escalate support to the right ventricle with the insertion of an Impella RP or the left ventricle with the insertion of larger devices, which provide more forward flow. Herein, we present a case of AMI complicated by cardiogenic shock resulting in biventricular failure treated with the percutaneous insertion of an Impella RP and Impella 5.0 utilizing invasive markers of left and right ventricular function to guide the management and escalation of care.


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