Left Ventricular Inflow Obstruction Research Articles

Blood Balloon Induced by an Atrial Myxoma in the Heart

A 75-year-old male with a left atrial (LA) mass was referred to our hospital. The patient had been healthy until 5 months prior, when dyspnea on exertion developed. A chest radiograph showed mild enlargement of the cardiac silhouette. An ECG showed sinus rhythm with P mitrale (Figure 1A). Transthoracic echocardiography (TTE) revealed a 5.4×3.9-cm hypoechoic tumor that had prolapsed through the mitral valve causing left ventricular inflow obstruction (Figure 1B; Movie I in the online-only Data Supplement). The tumor appeared to be soft with smooth surface and had a stalk originating from the interatrial septum. Transesophageal echocardiography (TEE) revealed dynamic smoke-like echoes, indicative of spontaneous echo contrast, inside the tumor (Figure 1C; Movie I in the online-only Data Supplement). M-mode showed multiple echoes and unique tumor behavior with subtle motion, in which the tumor rebounded into LA cavity twice during systole, producing an M-shaped signal (Figure 1D; Movie II in the online-only Data Supplement). These echocardiographic findings implied that the inside of tumor was filled with liquid, rather than clotted blood, giving it a blood-balloon–like appearance. Moreover, color Doppler revealed afferent flow entering the tumor (Figure 2A) and efferent flow draining from the tumor (Figure 2C). Both flows occurred …

Surgical correction of cor triatriatum sinister in the paediatric population: mid-term results in 15 cases.

Cor triatriatum sinister (CTS) is a rare developmental cardiac abnormality resulting in left ventricular inflow obstruction. In this report, we aimed to present our mid-term results of early childhood patients operated for CTS and associated cardiac abnormalities. We enrolled 15 patients with CTS who were operated by a single surgeon between 2001 and 2013. A retrospective analysis was performed in order to determine the demographics, operative and postoperative results of the patients. The median age was 14 months and the median body weight was 8.2 kg at the time of operation. Fourteen patients had concomitant cardiac pathology. Three of the patients had atrial septal defect and 1 of the patients had partial abnormal pulmonary venous connection, whereas 4 of the patients had both. In 2 cases of complete atrioventricular septal defect, 1 case with ventricular septal defect, 1 case with patent ductus arteriosus, 1 case with double outlet right ventricle and another case with tetralogy of Fallot, complete repair was performed together with membrane resection in the left atrium (LA). The mean preoperative left atrial gradient was 13.3 mmHg, whereas the mean LA pressure at the first postoperative year was 4.2 mmHg. There was 1 case with early mortality due to septic multiorgan failure secondary to pneumonia. CTS is a rare congenital cardiac anomaly in which the results of the corrective surgery are encouraging. Early and long-term outcomes may be variable according to the associated congenital heart defects.

Dynamic Left Ventricular Assist Device Inflow Obstruction

A 72-year-old man with a history of rheumatic heart disease, prior bioprosthetic mitral and aortic valve replacement, severe nonischemic dilated cardiomyopathy (left ventricular [LV] ejection fraction 5%), and a history of ventricular tachycardia (VT) underwent implantation of a continuous flow LV assist device (LVAD; HeartMate II; Thoratec Corporation) as destination therapy for severe heart failure. The early postoperative period was unremarkable and he had substantial functional improvement with minimal cardiovascular limiting symptoms after postoperative recovery. After ≈3 years on LVAD support, he experienced a series of appropriate implantable cardioverter-defibrillator shocks for sustained VT refractory to antiarrhythmic therapy. He underwent VT ablation targeting the basal LV inferoseptum, but he continued to have recurrent VT. Because VT seemed to originate from a deep septal substrate, he underwent an alcohol …

Long-Term Follow-Up of Neonatal Coarctation and Left-Sided Cardiac Hypoplasia

Neonatal coarctation of the aorta (CoA) is often associated with hypoplastic left-sided cardiac structures. Limited data are available regarding the long-term clinical outcomes. Our purposes were to assess the following in neonates with CoA and left-sided cardiac hypoplasia: (1) left-sided cardiac structure growth over time, (2) echocardiographic parameters predicting reintervention, and (3) long-term outcomes and reintervention rates. Data were collected for all patients born with isolated CoA, along with a mitral or aortic Z-score of <-2, who underwent repair before 2 months of age from January 1993 to April 1997. Recent follow-up data were available for 51 of 63 patients (81%) aged 15.4 ± 1.5 years (range 11 to 18). Of the 51 patients, 46 (90%) had a recent echocardiogram, all with normal left ventricular systolic function. The mitral and aortic valve annulus Z-scores increased significantly from the initial measurements: -3.29 ± 1.54 to -0.94 ± 0.58 (p <0.0001) and -3.25 ± 1.98 to 0.08 ± 1.81 (p <0.0001). No significant change was seen from intermediate (6.1 ± 1.6 years) to the latest follow-up examination. Of the 51 patients, 12 (24%) required reintervention at 3 ± 4.5 years; 9 for repeat CoA, 4 for subaortic stenosis, and 2 for aortic stenosis (some in combination). The initial echocardiographic parameters were not associated with reintervention. The overall freedom from reintervention was 76% at 15 years of follow-up. In conclusion, the long-term outcomes after isolated CoA repair with associated left-sided cardiac hypoplasia were excellent. Both aortic and mitral valve sizes had increased substantially by intermediate follow-up but tended to normalize afterward. Although 24% of the patients required reintervention, significant left ventricular inflow or outflow tract obstruction was uncommon.

Left atrial myxoma—influence of tumour size on electrocardiographic findings

ObjectiveThe data of 51 patients (33 females) who underwent excision of left atrial (LA) myxoma were retrospectively reviewed for correlation of tumour size and electrocardiographic (ECG) findings. Methods and resultsMean age was 39.1 ± 15 years (range 9–53 years). The LA enlargement (LAE) on ECG was defined by standard criteria. The LAE in ECG in these patients did not correlate with echocardiographic LA dimensions or with the degree of left ventricular (LV) inflow obstruction. But it was found that the presence of LAE in ECG predicted maximum tumour dimension of >5 cm and correlated with the degree of mitral regurgitation (MR). The LAE in ECG disappeared following surgery in 87.5% of patients. ConclusionThe LA enlargement on ECG in a patient with LA myxoma signifies larger tumour size or the presence of significant MR but is not necessarily associated with an increased LA size or LV inflow obstruction.

Congenital giant cardiac tumor with severe left-ventricular inflow and outflow obstruction and arrhythmia treated with pulmonary artery banding and long-term amiodarone infusion

We report a congenital giant cardiac tumor that occupied the majority of left ventricular cavity with severe left ventricular inflow and outflow obstruction. The hemodynamics were similar to univentricular physiology. He was treated with prostaglandins and bilateral pulmonary artery banding. He had frequent supraventricular tachycardia associated with ventricular pre-excitation that was controlled by long-term administration of intravenous amiodarone. The patient died due to sepsis after 3 months.

Extra-cardiac compression and left ventricular inflow obstruction as a complication of a Sengstaken–Blakemore tube

A 78-year-old man was admitted for a proximal humerus fracture after an accidental fall. On Day 4 after admission, he developed haematemesis. Oesophagogastroscopy revealed oesophageal varices. Bleeding could be initially controlled by an endoscopic ligature of the varices, but 1h later, recurrent haematemesis was seen. A Sengstaken–Blakemore tube was passed down the oesophagus and the gastric balloon was inflated inside the stomach. A traction of 1 …

A Case of Primary Unclassified Spindle Cell Sarcoma of the Left Atrium

Primary cardiac tumors are very uncommon. Seventy five percent of them are benign tumors and these are mostly myxomas. The malignant cardiac tumors, the majority of which are undifferentiated sarcomas, comprise up to 25% of all cardiac tumors. A primary malignant sarcoma of the myocardium is exceedingly rare. Thus, there have been very few such cases reported in the literature. We present here a case of a 15 year old man who had complaints of orthopnea and increasing exercise intolerance over a one month period. Transthoracic echocardiography demonstrated a well demarcated huge mass with left ventricular inflow obstruction on the posterior wall of the left atrium. The patient's symptoms were relieved by surgery. The histological diagnosis was an unclassified spindle cell sarcoma.

Effect of percutaneous mitral repair with the MitraClip® device on mitral valve area and gradient

Percutaneous repair of mitral regurgitation (MR) by leaflet apposition using a clip deployed via transseptal catheterisation is undergoing evaluation. In order to detect the potential for clinically significant left ventricular inflow obstruction after percutaneous repair, we measured mitral valve area (MVA) and mean transmitral gradient (MVG) echocardiographically in 96 patients implanted with a clip followed for up to 24 months. By planimetry, the mean MVA decreased from 6.0 +/- 1.3 cm2 to 3.6 +/- 1.2 cm2 (p < 0.05) (range 1.9 to 7.6 cm2) after clip placement, and remained unchanged after 24 months of follow-up (3.5 +/- 0.8 cm2). The mean MVG increased after clip placement from 1.7 +/- 0.9 mmHg to 4.1 +/- 2.2 mmHg (p < 0.05), and did not increase further to 24 months (3.8 +/- 1.9 mmHg). There were no differences in MVA or MVG between patients who received 1-clip (69%) and those receiving 2-clips (31%). Patients with functional MR (23%) had a slightly smaller MVA, both at baseline and after clip placement, but did not differ from degenerative MR patients at later follow-up. After 2 years of follow-up, no patient required surgery for LV inflow obstruction. Mitral repair with the MitraClip device for MR decreases MVA without significant mitral obstruction. After 2 years of follow-up, no patient required surgery for LV inflow obstruction, and these results were not influenced by the use of more than 1 clip or the aetiology of MR.

Cor Triatriatum Associated with ASD and Common Atrium in 7-Month-Old With Tachypnea and Failure to Thrive

A 7-month-old child without significant past medical history was presented to her primary care physician with progressive symptoms of tachypnea and failure to thrive. A transthoracic echocardiogram revealed an atrial septal defect measuring 26 mm essentially creating a common atrium. Also observed was an interruption of the inferior vena cava with azygous continuation. Operative intervention was scheduled. Of note, the patient's twin sister underwent repair of an atrioventricular septal defect as an infant. The intraoperative transesophageal echocardiogram is shown in Figure 1 (* = redundant fenestrated membrane), and the transesophageal echocardiogram apical 4-chamber view is shown in Figure 2 (left atrium [LA], left ventricle [LV], mitral valve [MV], right atrium [RA], right ventricle [RV], arrow = redundant fenestrated membrane), which again revealed essentially a common atrium. In addition, there was a redundant membrane superior to the mitral valve resulting in a two-chambered left atrium without obstruction, consistent with cor triatriatum sinister.Fig 2View Large Image Figure ViewerDownload (PPT) The atrial septal defect was approached through a standard right atriotomy after institution of cardiopulmonary bypass. The redundant fenestrated left atrial membrane was identified. Consistent with the intraoperative transesophageal echocardiographic findings, this fenestrated membrane was observed to be proximal (superior) to the mitral valve with insertion to the remnant of primum septum as seen in Figure 2. The pulmonary veins were noted to empty into the proximal atrial chamber, whereas the ostium of the left atrial appendage was distal to THE insertion of the membrane as demonstrated in Figure 3 (arrow = redundant fenestrated membrane above the mitral valve; left atrial appendage [LAA]). On inspection, the membrane was found to be fibrinous tissue reminiscent of valvular tissue. The membrane was completely excised from its attachments, followed by patch closure of the atrial septal defect with autologous pericardium. The postoperative transesophageal echocardiography showed no valvular insufficiency or residual atrial septal defect. Cor triatriatum represents less than 0.1% of all congenital cardiac malformations. In its most common form, cor triatriatum sinister, the left atrium is divided into a proximal and distal chamber, with a membrane partitioning the left atrium, with attachment to the atrial septum. We believe that no other description of cor triatriatum with common atrium in the pediatric population has been published. The anatomy of this patient was unique due to both the lack of atrial septum and the site of attachment of the membrane. This membrane fits the definition of cor triatriatum, with the membrane inserting above the annulus of the mitral valve and extending superior to the ostium of the left atrial appendage. When grossly viewing the membrane intraoperatively, we initially believed that the membrane was a supra-mitral ring, until the extent of the attachments was elucidated. Interestingly, this membrane was not clearly defined and was interrogated on two prior transthoracic echocardiograms, but as noted in Fig 2, Fig 3, the membrane was clearly visible in the intraoperative transesophageal echocardiography. In retrospect, this finding was indeed present on the preoperative transthoracic echocardiogram, but it was not obvious to the referring cardiologist. Without intraoperative transesophageal echocardiogram modality, we would have most likely missed this cor triatriatum membrane, which would have likely led to an increased mid-term and long-term risk for development of left ventricular inflow obstruction.

Asymptomatic Malaria Associated with Protection: Not Causal

An otherwise healthy 44-year-old woman (a resident of a mountainous region of Switzerland) presented to the emergency department with acute onset of fever myalgia and disorientation. On physical examination she was in shock without an evident infectious source. Laboratory analyses revealed leukocytosis (WBC count 27000_109 cells/L) and an elevated C-reactive protein level (260 mg/ L). The patient had a history of congenital left ventricular inflow and outflow tract obstruction that was surgically corrected at the age of 15 years. In 1986 and 1996 she had infectious endocarditis of the aortic valve due to Staphylococcus aureus; the aortic valve was replaced by a homograft in 1990. Transesophageal echocardiography showed vegetations on the aortic valve and possibly also on the mitral valve with severe aortic and moderate mitral regurgitation. Therapy with vancomycin gentamicin and rifampicin was initiated. Blood cultures grew methicillin-sensitive S. aureus supporting a diagnosis of S. aureus prosthetic valve endocarditis and treatment was changed to flucloxacillin and rifampicin. The clinical course was complicated by acute renal failure severe thrombopenia and multiple septic emboli (in the brain skin retina and spleen). After 4 weeks of antibiotic therapy the aortic and mitral valves were replaced by mechanical prostheses. Cultures of the aortic valve were sterile but broad-range eubacterial PCR had results positive for Coxiella burnetii. Serologic results confirmed chronic Q-fever (phase 1 IgG titer 1:6400; phase 1 IgA titer 1: 200; phase 2 IgG titer 1:12800; and phase 2 IgA titer 1:400); analysis of stored serum samples from the patient revealed that the infection had been acquired in the previous 12 months. Thus the final diagnosis was chronic Q-fever prosthetic valve endocarditis complicated by acute S. aureus prosthetic valve endocarditis. The postoperative course was uncomplicated. S. aureus prosthetic valve endocarditis was treated for a total of 6 weeks with flucloxacillin and rifampicin. For Q-fever endocarditis therapy with doxycycline and hydroxychloroquine was introduced and will be continued until the phase 1 IgG titer is !1:400 with a minimum treatment duration of 18 months. At the most recent follow-up visit 10 months after the initial presentation the patient was asymptomatic and titers of anti-Coxiella antibodies were decreasing (phase 1 IgG titer 1:3200; phase 1 IgA titer 1:50; phase 2 IgG titer 1: 6400; phase 2 IgA titer 1:100). This case is remarkable for the simultaneous implication of two different bacterial species in the pathogenesis of infective endocarditis with one of the pathogens being discovered incidentally. Reports in the literature on dual-pathogen endocarditis are scarce; 2 cases with concomitant streptococcal and Q-fever endocarditis have been previously described in a large prospective French study [1]. Our patient had underlying cardiac abnormalities predisposing her to C. burnetii endocarditis and resided in an area of Switzerland with a seroprevalence of Coxiella species infection up to 30% [2]. Nevertheless C. burnetii infection was not suspected because all clinical manifestations were compatible with staphylococcal prosthetic valve endocarditis. The diagnosis of Q-fever endocarditis was thus a lucky strike which is disconcerting given the poor prognosis of unrecognized chronic Coxiella infection. In view of our experience we believe that C. burnetii serologic analysis or PCR testing of heart valve tissue are worth performing in all patients with infective endocarditis who have a preexisting valvulopathy and live in a region in which Q-fever is endemic regardless of whether another etiological agent has been identified or not. (full-text)

Live three-dimensional echocardiography of cor triatriatum in a child

Cor triatriatum sinistrum is a very rare condition constituting 0.1–0.4% of congenital heart disease. Patients most commonly present with breathlessness, and may be falsely diagnosed with asthma. Pulmonary hypertension may...

Prolapsing left atrial myxoma: preoperative diagnosis using a multimodal imaging approach with magnetic resonance imaging and real-time three-dimensional echocardiography

Real-time three-dimensional echocardiography (RT3DE) is a new promising technique for the evaluation of intracardiac masses. We present the diagnostic work-up using a multimodal-imaging approach in a 74-year-old patient with a prolapsing tumour in the left atrium suggestive of a myxoma, causing severe congestive heart failure attributable to dynamic left ventricular inflow obstruction, and mimicking severe mitral valve stenosis. Real-time three-dimensional echocardiography allowed to accurately image the entire volume of the myxoma, and to analyse the dynamic left ventricular inflow obstruction. The size of the lobulated mass as assessed by RT3DE was 65 x 25 x 22 mm. The mass was surgically removed, histology was diagnostic for myxoma, and the patient had an uneventful recovery. Real-time three-dimensional echocardiography images the entire volume of a mass allowing for accurate measurements in multiple planes, and allowing for real-time evaluation of obstructive effects on ventricular in- or outflow. This case shows how RT3DE and other non-invasive imaging modalities may be used as complementary techniques for evaluation of intracardiac masses.

Pseudoaneurysm With Thrombus and Left Ventricular Inflow Obstruction After Left Circumflex Stenting

A 61-year-old woman had stenting of the left circumflex coronary artery. She had a repeat coronary angiogram the day after stenting because of hypotension and orthopnea. The left circumflex stent was patent. A transesophageal echocardiogram showed a 2.5 cm x 3.0-cm mass in the atrioventricular groove compressing the left atrium. A pseudoaneurysm with thrombus and left ventricular inflow obstruction was diagnosed. The patient was observed for 48 hours to allow the pseudoaneurysm to seal and coagulate. She then had surgical evacuation of the thrombus, which had caused her hypotension and orthopnea by compression of the left atrium.

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.

Invited commentary

Nowadays in congenital cardiac surgery it is unusual to discover new pathologies. However, a sum of “minor” circumstances could create new entities that may need to be treated. In my opinion this is the case when a persistent left superior vena cava (LSVC) appears to be draining in a significantly dilated coronary sinus. This association has been proven to be partly responsible for significant left atrioventricular supravalvar obstruction. An excessive flow along the posterior inferior left atrial wall in itself may markedly enlarge even a “normal” LSVC with a normal coronary sinus up to a point of partial obstruction of left ventricular inflow.

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.

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.