Ventricular Inflow Obstruction Research Articles

Dynamic Left Ventricular Inflow Obstruction Associated with a Left Atrial Myxoma

A 49-year-old female presented with lower extremity weakness, worsening dyspnea, stigmata of systemic embolism, and an acute episode of dysarthria. Brain computed tomography and magnetic resonance imaging demonstrated small embolic strokes while transthoracic echocardiographic examination showed a large left atrial (LA) mass. The patient was scheduled for surgical excision of the mass. Intraoperative two-dimensional and three-dimensional transesophageal echocardiography (TEE) (Siemens Sequoia, Mountain View, CA and Tom Tek Corporation, Boulder, CO) demonstrated a 4 × 6 cm2 pedunculated LA mass attached by a pedicle to the superior fossa of the interatrial septum [Fig. 1 and Video Loop 1 (Please see video loops available at www.anesthesia-analgesia.org)]. During diastole, the mass prolapsed through the mitral valve resulting in a turbulent color flow Doppler pattern (Video Loop 2). Continuous wave doppler revealed mild to moderate left ventricular (LV) inflow obstruction, with calculated mean and peak pressure gradients of 6 mm Hg and 9 mm Hg, respectively (Fig. 2). In general, mild mitral obstruction is suggested by a mean pressure gradient <6 mm Hg, moderate obstruction by a mean pressure gradient of 6–10 mm Hg, and severe obstruction by a gradient >10 mm Hg. Proximal isovelocity surface area can provide information about the degree of obstruction, but the asymmetry of the proximal isovelocity surface area hemisphere in this case may have resulted in an inaccurate estimate of the diminished LV inflow area. The use of pressure half-time is also commonly used to estimate the severity of mitral obstruction, however, this technique has not been validated in patients with dynamic LV inflow obstruction.Figure 1.: Intraoperative two-dimensional transesophageal mid-esophageal four-chamber view demonstrating the left atrial myxoma (arrow) prolapsing through the mitral valve during diastole. LA: left atrium; LV: left ventricle; RV: right ventricle.Figure 2.: Intraoperative continuous wave Doppler image from the transesophageal mid-esophageal four-chamber view demonstrating the left ventricular inflow velocity profile along the prolapsing left atrial myxoma. Pressure gradients (ΔP) calculated from the modified Bernoulli Equation (ΔP = 4V 2; V = peak velocity), were obtained from values averaged over three cardiac cycles. The calculated mean (6 mm Hg) and peak (9 mm Hg) pressure gradients are consistent with mild-to-moderate obstruction.A complete excision of the LA mass and its atrial septal attachment was performed and the resultant atrial septal defect was closed with pericardial patches. After cardiopulmonary bypass, TEE confirmed an intact interatrial septum. Pathologic examination confirmed the mass to be a myxoma. The patient recovered uneventfully and was discharged home on postoperative Day 5. Seventy-five percent of myxomas are found in the LA, but 10–20% originate in the right atrium, and 5% in either ventricle. In contrast to this report, LA myxomas typically attach via a narrow base to the interatrial septum at the border of the fossa ovalis, and are rarely invasive. Patients with myxomas who become symptomatic usually present with congestive heart failure (67%) because of LV inflow obstruction, signs of tumor embolization (29%), or constitutional symptoms (41%) (1,2). Intraoperative TEE is used for identifying satellite tumors and confirming the structural integrity of the interatrial septum after surgical excision of a myxoma (3). Three-dimensional TEE may be helpful for delineating the extent of the myxoma's attachment, thereby permitting more accurate surgical planning.

Cor Triatriatum Sinister with and without Left Ventricular Inflow Obstruction: Visualization of the Entire Supravalvular Membrane by Real-time Three-dimensional Echocardiography. Impact on Clinical Management of Individual Patient

We present 4 cases of cor triatriatum in whom the diagnosis was correctly made by 2-dimensional transthoracic echocardiography, which showed the supravalvular left atrial membrane that divides the left atrium into 2 chambers. The pulmonary veins were connected normally to the proximal left atrial chamber and the left atrial appendage was connected to the distal left atrial chamber. In 1 patient there was evidence of severe pulmonary venous obstruction to the mitral valve by Doppler examination, while in the other three, there was no venous obstruction. Patients were then examined by real-time 3-dimensional echocardiography (RT3DE, using x4 matrix array transducer connected to Sonos 7500 echocardiographic system Phillips, Andover, Mass, USA). This showed the exact morphology of the membrane and led to cancellation of planed surgical intervention in 1 case in which the membrane was only a broad band crossing the left atrial cavity. In addition to delineating the exact morphology of the intracavitary anomaly, this novel echocardiographic imaging modality should be an additive tool to better understand the natural history of these nonobstructive left atrial membranes via longitudinal follow-up of these patients.

Surgical Approach to Left Ventricular Inflow Obstruction Due to Dilated Coronary Sinus

Left superior vena cava draining to a dilated coronary sinus can cause left ventricular inflow obstruction. Our purpose is to report 4 severely ill patients with this malformation who were operated upon and in whom repair was accomplished using an original surgical approach. An operative procedure was designed, which included complete resection of the wall of the coronary sinus along its entire extension in the left atrium; division of the left superior vena cava; and establishment of the left superior vena cava-right atrial continuity by a wide left superior vena cava-right atrial appendage anastomosis. The series included 1 patient with interrupted inferior vena cava-hemiazygous continuation to left superior vena cava. There were no deaths. Absence of residual left ventricular inflow obstruction was demonstrated at follow-up in all cases, together with an unobstructed left superior vena cava-right atrial appendage-right atrial connection. A predictable relief of the left ventricular inflow obstruction, together with preservation of an adequate drainage for the systemic venous return, were both achieved with this repair.

Current Management of Severe Congenital Mitral Stenosis

Severe congenital mitral stenosis (MS) is a rare anomaly that is frequently associated with additional left heart obstructions. Anatomic treatments for congenital MS include balloon mitral valvuloplasty (BMVP), surgical mitral valvuloplasty (SMVP), and mitral valve replacement (MVR), although the optimal therapeutic strategy is unclear. Between 1985 and 2003, 108 patients with severe congenital MS underwent BMVP or surgical intervention at a median age of 18 months (range 1 month to 17.9 years). Anatomic subtypes of MS were "typical" congenital MS in 78 patients, supravalvar mitral ring in 46, parachute mitral valve in 28, and double-orifice mitral valve in 11, with multiple types in approximately 50% of patients. Additional left heart anomalies were present in 82 patients (76%). The first MS intervention was BMVP in 64 patients, SMVP in 33, and MVR in 11. BMVP decreased peak and mean MS gradients by a median of 33% and 38%, respectively (P<0.001), but was complicated by significant mitral regurgitation in 28%. Cross-sectional follow-up was obtained at 4.8+/-4.2 years. Overall, Kaplan-Meier survival was 92% at 1 month, 84% at 1 year, and 77% at 5 years, with 69% 5-year survival during the first decade of our experience and 87% since (P=0.09). Initial MVR and younger age were associated with worse survival. Survival free from failure of biventricular repair or mitral valve reintervention was 55% at 1 year among patients who underwent BMVP and 69% among patients who underwent supravalvar mitral ring resection initially. Among patients who underwent BMVP, survival free from failure of biventricular repair or MVR was 79% at 1 month and 55% at 5 years, with worse outcome in younger patients and those who developed significant postdilation mitral regurgitation. BMVP effectively relieves left ventricular inflow obstruction in most infants and children with severe congenital MS who require intervention. However, surgical resection is preferable in patients with MS due to a supravalvar mitral ring. Five-year survival is relatively poor in patients with severe congenital MS, with worse outcomes in infants and patients undergoing MVR, but has improved in our more recent experience. Many patients have undergone second procedures for either recurrent/residual MS or mitral regurgitation resulting from dilation-related disruption of the mitral valve apparatus.

Emergency Management of Severe Right Ventricular Inflow Obstruction Secondary to a Metastatic Cardiac Tumor

We report the case of a 21-year-old female with previous history of primitive neuroectodermal tumor of the anterior abdominal wall who developed severe manifestations of right heart failure due to a mass obliterating the right ventricular cavity. She underwent emergent resection of the mass with histopathologic confirmation of metastatic neuroectodermal tumor. To the best of our knowledge, this is the first case of emergent surgical excision of a metastatic cardiac tumor of primitive neuroectodermal origin.

Left ventricular inflow obstruction associated with persistent left superior vena cava and dilated coronary sinus

BackgroundIt has previously been suggested that significant dilatation of the coronary sinus can contribute to left ventricular inflow obstruction and is amenable to surgical correction. The purpose of this study was to review our experience with this rare condition. MethodsSince 1995, 6 patients have undergone coronary sinus reduction for concerns of obstruction with other concomitant intracardiac repairs. Preoperative echocardiography identified a significantly dilated left superior vena cava to the coronary sinus in 5 patients (83%) and an abnormal mitral valve in 4 patients (67%); these resulted in abnormal Doppler inflow patterns. Preoperative cardiac catheterization was performed in 5 patients and revealed increased atrial “a” waves, with a gradient to the left ventricular end-diastolic pressure in each case. At the time of surgery, coronary sinus angioplasty was performed in all patients. ResultsThere were no deaths, and there was no major morbidity. Follow-up imaging revealed no significant left ventricular inflow obstruction in any patient. ConclusionsWe conclude that dilatation of the coronary sinus can become hemodynamically significant and that coronary sinus angioplasty is a safe and effective technique.

Right ventricular hemangioma causing right ventricular inflow obstruction and right heart failure

We report a patient who presented with symptoms of right heart failure. Transesophageal echocardiography revealed a right ventricular mass, causing right ventricular inflow obstruction. Coronary angiography revealed a characteristic tumor blush. After successful surgical resection, histologic findings were consistent with a right ventricular hemangioma.

Primary cardiac carcinosarcoma: a rare, aggressive tumor

Primary cardiac carcinosarcoma is a rare cardiac tumor, and little is known about its natural history. We treated a man who had no evidence of a cardiac mass at initial echocardiography, but 19 months later he had a large biatrial tumor with left ventricular inflow obstruction. Only palliative resection could be achieved. Four months postoperatively extensive tumor recurrence was found, and he died a month later. Histologic examination showed cells with features of both rhabdomyosarcoma and adenocarcinoma, and the tumor was classified as a primary cardiac carcinosarcoma.

Extension of a Pulmonary Blastoma Into the Left Atrium

Extension of a Pulmonary Blastoma Into the Left Atrium

Aneurysmal pericardial patch producing right ventricular inflow obstruction

A 2-month-old infant presented with acute onset of heart failure, having previously undergone anatomical repair of transposition of the great arteries and ventricular septal defect (VSD). Echocardiography demonstrated aneurysmal dilation of the native pericardial patch used for VSD closure, resulting in right ventricular inflow obstruction. The pericardial patch was excised, and the VSD closed using a GoreTex patch.

Prosthetic Mitral Valve Replacement: Late Complications After Native Valve Preservation

Preservation of the mitral valve leaflet and tensor apparatus during valve replacement is believed to maintain left ventricular performance. The routine use of this technique may lead to left ventricular outflow or inflow obstruction as illustrated in the present report. We recommend mobilization or excision of the anterior mitral valve leaflet and preservation of the posterior leaflet if replacement of the valve is contemplated for incompetence.(Ann Thorac Surg 1997;63:541–3)Accepted for publication Oct 15, 1996.

Echocardiographic evaluation of congenital mitral valve anomalies in children

Congenital mitral valve anomalies were diagnosed in 65 children, whose ages ranged from newborn to 18 years, using 2-dimensional, color, pulsed-, and continuous-wave Doppler ultrasound. Data were collected over 7.5 years from 13,400 new studies. Data in these patients were compared with those obtained by cardiac catheterization, cardiac surgery, and autopsy. We detected 4 different lesions: (1) congenital mitral stenosis with 2 papillary muscles (n = 24); (2) parachute mitral valve, with a single papillary muscle (n = 24); (3) isolated cleft in the mitral valve (n = 10); and (4) double-orifice mitral valve (n = 7). A supravalvar mitral ring was detected in 21 patients with mitral stenosis; however, it never occurred as an isolated lesion and was invariably associated with some other left ventricular inflow or outflow obstruction. The supravalvar ring was associated with a parachute deformity of the mitral valve in 17 patients; in only 4 was this abnormality associated with mitral stenosis with 2 papillary muscles. In patients with congenital mitral stenosis, the peak and mean transmitral Doppler velocities were increased significantly compared with those in controls (peak velocity 1.53 ± 0.74 vs 0.86 ± 0.25 m/s, respectively, p < 0.01; mean velocity 1.13 ± 0.61 vs 0.58 ± 0.11 m/s, respectively, p < 0.01). The correlation between mean transmitral pressure gradient obtained by Doppler and cardiac catheterization was fair (r = 0.75). However, the correlation between the mitral valve areas calculated by the Doppler pressure half-time method and by the Gorlin formula was poor (r = 0.57). Eleven of the 24 patients with parachute mitral valve deformities had Doppler evidence of an increased transmitral gradient. In children, echocardiography proved superior to cineangiography as a diagnostic modality, and can be relied upon in making management decisions, but Doppler velocities, including pressure halftime, applicable in adults for calculation of mitral valve area are unreliable.

Intracardiac extension of a lung tumor causing left ventricular inflow obstruction

A patient with the diagnosis of small cell lung cancer had syncopal episodes. The tumor was found to invade the right upper pulmonary vein with extension into the left atrium. The mass was protruding across the mitral valve producing ball-valve blockade. In this report the echocardiographic signs of a primary lung tumor with intraatrial extension causing left ventricular inflow tract obstruction are described.

Cor triatriatum and rheumatic mitral stenosis in adulthood: Assessment of combined congenital and acquired cardiac disease in a 60-year-old woman

Cor triatriatum is a congenital anomaly rarely described in adults. It causes left ventricular inflow obstruction and mimics mitral stenosis. The 2 conditions have rarely been reported concurrently. This report describes the case of a 60-year-old women with rheumatic mitral stenosis and single vessel coronary disease, in whom cor triatriatum was diagnosed coincidentally at echocardiography. All lesions were successfully managed by surgery.

The levoatriocardinal vein: Morphology and echocardiographic identification of the pulmonary—systemic connection

This study considers the array of pulmonary-systemic connections made by the levoatriocardinal vein. The primary and associated lesions that play a role in forming this vein are examined, and echocardiography is discussed as a method for its rapid identification. The levoatriocardinal vein is a pulmonary-systemic connection that provides an alternative egress for pulmonary venous blood in left-sided obstructive lesions. It is thought to result from the persistence of anastomotic channels that connect the capillary plexus of the embryonic foregut to the cardinal veins. Only 12 cases of levoatriocardinal vein have been reported since its first description in 1926. A comprehensive description of the morphology and echocardiographic identification of this lesion has been unavailable because of its rarity. A retrospective study was performed in 13 patients with a levoatriocardinal vein from the University of California, San Francisco. Echocardiographic findings were compared with those obtained by angiography or at necropsy. In addition, the details of 12 previously published case reports were reviewed. Age at presentation, primary obstruction to pulmonary venous return, integrity of the atrial septum and origin and drainage of the levoatriocardinal vein were compared. Patient age at presentation was < 2 years, with most patients presenting before age 6 months. Variations of the hypoplastic left heart syndrome accounted for the majority of primary defects encountered, although multiple but less severe left-sided lesions were seen. The atrial septum was functionally intact in most patients. The levoatriocardinal vein, defined echocardiographically, originated predominantly from the smooth-walled left atrium and drained to the superior vena cava or innominate vein; however, variations of this pattern existed. As a physiologic entity, the levoatriocardinal vein provides a mechanism for decompression of pulmonary venous return primarily in patients with left ventricular inflow obstruction. A levoatriocardinal vein is thought to form when the atrial septum fails to provide an alternate egress for left atrial blood. However, when a septal defect or alternative shunt occurs in conjunction with a levoatriocardinal vein, the clinical presentation may be postponed. Echocardiography provides a rapid, noninvasive modality for identifying the pulmonary-systemic connection, which may masquerade as the vertical vein in anomalous pulmonary venous connection or act as an occult source of left to right shunting in patients undergoing surgery for hypoplastic left heart syndrome.

Color flow Doppler determination of transmitral flow and orifice area in mitral stenosis: Experimental evaluation of the proximal flow-convergence method

To evaluate the in vivo accuracy of color Doppler flow-convergence methods for determining transmitral flow volumes and effective orifice areas in mitral stenosis, we studied two models for flow-convergence surface geometry, a hemispheric (HS) model and an oblate hemispheroid (OH) model in a chronic animal model with quantifiable mitral flows. Color Doppler flow mapping of the proximal flow-convergence region has been reported to be useful for evaluation of intracardiac flows. Flow-convergence methods in patients with mitral stenosis that use HS assumption for the isovelocity surface have resulted in underestimation of actual flows. Chronic mitral stenosis was created surgically in six sheep with annuloplasty rings (group 1) and in 11 sheep with bioprosthetic porcine valves (group 2). Hemodynamic and echocardiographic/Doppler studies ( n = 18 in group 1; n = 21 in group 2) were performed 20 to 34 weeks later. Left ventricular inflow obstruction was of varied severity, with mean transmitral valve gradients in group 1 ranging from 1.3 to 18 mm Hg and in group 2 ranging from 6.3 to 25.6 mm Hg. Although transmitral flows derived by both geometric flow convergence models showed significant correlations with reference cardiac outputs, the correlations for the OH model were better than those for the HS model (group 1, r = 0.86 for the OH model vs r = 0.72 for the HS model; group 2; r = 0.84 for the OH model vs r = 0.62 for the HS model). The OH model was also superior to the HS model in determining effective orifice areas compared to reference orifice areas determined by postmortem planimetry of anatomic orifices (group 1 only, r = 0.64 for OH vs 0.58 for HS), by the Gorlin and Gorlin formula (group 1, r = 0.63 for OH vs 0.72 for HS; group 2, r = 0.82 for OH vs 0.76 for HS), and by the Doppler pressure half-time method (group 1, r = 0.76 for OH vs 0.69 for HS; group 2, r = 0.84 for OH vs 0.62 for HS). The percentage differences between the reference values and calculated data with the OH model were significantly smaller for transmitral flows (−5.8% vs −59% in group 1 and −13% vs −63% in group 2) and for effective orifice areas in both groups, p < 0.0001 (−21% vs −56% for mitral orifice area by postmortem planimetry in group 1; −28% vs −61% in group 1 and −24% vs −68% in group 2 for mitral orifice area by the Gorlin and Gorlin formula; and −18% vs −57% in group 1 and −27% vs −71% in group 2 for the pressure half-time method, p < 0.0001 for each comparison). These studies demonstrate that flow convergence principles applied to color Doppler flow mapping permit estimation of transvalvular flow volumes and orifice areas in mitral stenosis. However, in the presence of orifices that are not infinitesimally small and when it is not clinically feasible to sample at substantial distances proximal to the orifice, determination of flows and areas with flow convergence principles results in underestimation relative to reference standards. This underestimation can be minimized by applying an OH geometric model to the flow convergence surface area rather than a strictly HS model.

Can a dilated coronary sinus produce left ventricular inflow obstruction? An unrecognized entity

Drainage of a persistent left superior vena cava to the coronary sinus is a common congenital lesion associated with dilatation of the coronary sinus. We report on 4 cases in which marked enlargement of the coronary sinus was present, immediately superior to and partially occluding the mitral valve, with consequent obstruction to left ventricular inflow. In 1 patient, presenting with cardiac failure in early infancy, this abnormality was the major defect, mimicking the features of cor triatriatum, and surgical correction led to significant symptomatic improvement. In 3 other patients this abnormality was associated with other congenital lesions, and it is postulated that in these cases impairment of flow into the left ventricle in the presence of an atrial septal defect elevated the left atrial pressure and increased the shunt across the atrial septum, thereby increasing the symptoms and clinical findings. Surgical reduction of the enlarged coronary sinus was performed in each case through the interatrial septum, and the technique is described.

Transesophageal Color Flow Echocardiographic Evaluation of Supravalve Mitral Ring in an Adult Patient

Supravalve mitral ring (SVMR) is an extremely rare form of congenital left ventricular inflow obstruction in which a left atrial membrane causes an obstruction keeping the left atrium from emptying into the left ventricle. Cardiac catheterization and angiography are not reliable means to diagnose this lesion. Transthoracic echocardiography (TTE) has proved to be the most sensitive tool for diagnosis of this form of left ventricular inflow obstruction; however, its diagnostic accuracy has been recently questioned. We report the complementary roles of TTE and transesophageal echocardiography in a case of SVMR in an adult patient. (ECHOCARDIOGRAPHY, Volume 11, May 1994)

Neonatal pneumomediastinum with isolated mitral obstruction.

While mediastinal air accumulation in the ventilated newborn is usually a benign entity, tension pneumomediastinum can embarrass both respiratory and cardiac function. A case of isolated left ventricular inflow obstruction caused by a pneumomediastinum in a premature infant is described that resolved with high frequency ventilation. The development of pneumomediastinum and haemodynamic compromise in a premature infant warrants echocardiographic examination.

Giant right atrial thrombus causing right ventricular inflow and outflow obstruction

Giant right atrial thrombus causing right ventricular inflow and outflow obstruction