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Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder in which there is faulty development of the arteries. There is a high incidence of pulmonary hypertension (PH) in these patients, the pathophysiology of which is not fully known. An increase in cardiac output, causing high-output cardiac failure, and increased pulmonary vascular resistance secondary to genetic mutations are the main reasons. We report a 25-year-old male with HHT who presented with right heart failure secondary to PH in whom both the above mechanisms were operating. The coexistence of giant pulmonary arteriovenous malformations with severe PH is a rare scenario influencing management decisions that are discussed. In addition, this patient highlights the classical visceral vascular malformations in this rare disorder.

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Unruptured sinus of Valsalva aneurysm can present with manifestations due to local compression or protrusion into the chambers. Right ventricular inflow obstruction is a rare manifestation. This image highlights the tricuspid valve obstruction due to a Valsalva aneurysm in a patient after surgical aortic valve replacement.

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Ascending aortic pseudoaneurysm is a known complication of previous cardiac surgery that needs surgical or endovascular intervention. The presence of arch branches complicates the later approach necessitating additional procedures, either hybrid surgical or endovascular modification of the grafts. We describe a patient who developed an ascending aortic pseudoaneurysm after mitral valve replacement, which recurred after surgical patch closure with the formation of a cutaneous fistula. This high-risk patient was managed by an endovascular approach with in-situ fenestration for the innominate artery by a simple technique.

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Bradyarrhythmia requiring pacing is infrequently encountered in patients with complex cyanotic congenital heart disease. Even though epicardial pacing is the preferred mode, rarely, a need for endocardial lead implantation arises. Patients with cavopulmonary shunts limit access to the venous atria and ventricles, necessitating alternate methods of pacemaker implantation. We report transvenous endocardial lead implantation by an unconventional method in a patient with congenitally corrected transposition of great arteries after a bidirectional Glenn shunt.

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An atrial septal defect is a rare anomaly in patients with interrupted inferior vena cava, which renders the percutaneous intervention more complex; and hence, innovative approaches should be sought. Dextrocardia further complicates the procedure, and traditional atrial septal device deployment methods cannot be employed. We report a successful percutaneous secundum atrial septal defect closure by a novel deployment strategy along with balloon dilation of associated severe valvular pulmonary stenosis in a patient with dextrocardia and interrupted inferior vena cava.

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BACKGROUND: The objective is to assess whether the squaring of tricuspid regurgitation velocity (TRV) gives an improved estimate of pulmonary vascular resistance (PVR) or is equivalent to the ratio of TRV and time velocity integral of right ventricular outflow tract (TVIRVOT) (TRV/TVIRVOT) for assessing PVR in patients with high PVR values. METHODS: Thirty patients predicted to have PVR >6 WU by Doppler were included in the present study. TRV and TVIRVOT were measured by echo Doppler. TRV/TVIRVOT and TRV2/TVIRVOT were calculated. PVRCATH was estimated within 2 h of Doppler study. Regression equations for calculating PVR from TRV/TVIRVOT (PVRECHO1) and TRV2/TVIRVOT (PVRECHO2) were developed. Bland-Altman analysis for agreement between PVRCATH and PVRECHO1, PVRECHO2 was carried out. RESULTS: The mean value of PVRCATH was found to be 15.08 ± 7.03 WU. The calculated values of PVRECHO1 and PVRECHO2 were found to be 15.08 ± 6.34 WU and 15.05 ± 6.08 WU, respectively. The linear regression analysis carried out for PVRCATH and TRV/TVIRVOT showed good correlation (R = 0.84). Bland-Altman analysis showed excellent agreement between the two Doppler methods and invasive PVR with negligible bias. CONCLUSION: Noninvasive estimation of PVR by Doppler is reliable even in patients with high PVR (>6 WU) and, squaring TRV is not superior to TRV alone.

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OBJECTIVE: To document changes in pulmonary arterial systolic pressure (PASP) in patients with SLE who have received CYC for any indication. METHODS: Twenty-four patients with SLE pulmonary arterial hypertension (PAH) with a PASP of >30 mmHg by transthoracic echocardiography received i.v. CYC (n = 24) or steroids (n = 24) with or without vasodilators (n = 20). Baseline clinical characteristics and PASP were evaluated before and after therapy at 6 months. Responders were defined as those who had a decrease in PASP of >15 mmHg from baseline along with improvement in their New York Heart Association functional class. RESULTS: There were 11 responders (45.83%), with a decrease in mean PASP from 59.33 mmHg at baseline to 43.29 mmHg at the end of 6 months (P < 0.0001). The decrease in mean PASP from 39.75 mmHg at baseline to 34.4 mmHg at the end of 6 months was significant in four patients who received immunosuppression alone (P = 0.04). There was no difference in baseline PASP and disease activity between responders and non-responders. Two deaths were noted. CONCLUSION: Immunosuppression and vasodilators produced significant improvement in SLE PAH over 6 months.

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BACKGROUND: Pulmonary vascular resistance (PVR) is a crucial parameter in the management of patients with left-to-right shunt lesions. Cardiac catheterization (Cath) is the gold standard test to assess PVR (PVRcath ), but it is invasive and hence, risky in children with pulmonary arterial hypertension (PAH). A noninvasive tool to assess PVR is desirable. Ratio of tricuspid regurgitation velocity (TRV) and time-velocity integral of right ventricular outflow tract (TVIRVOT ) by Doppler was previously shown to be a reliable noninvasive method for estimation of PVR in acquired PAH. METHODS: Peak TR velocity and TVIRVOT were recorded from 63 prospective patients with various congenital shunt lesions. Subsequently, the patients were subjected to cath in less than 2 hours. The patients were subdivided into four subsets based on age and pulmonary arterial mean pressure (PAMP). A regression equation was developed for calculation of PVR from TRV/TVIRVOT (PVREcho ) which was indexed for BSA (PVRIEcho ). Bland-Altman analysis was done for agreement between PVRIcath and PVRIEcho . Receiver operating characteristic (ROC) curves were plotted to test the identity of the two methods and also the applicability of PVRIEcho across a wide range of Wood units. RESULTS: Receiver operating characteristic curve plotted between the two methods showed good identity. Bland-Altman analysis showed excellent agreement between the two methods with negligible bias. ROC curves showed that PVRIEcho was accurate in distinguishing different cutoff values of PVR in each of the 4 groups. CONCLUSION: Noninvasive Doppler estimation of PVR is reliable in patients with shunt lesions over a wide range of PVR.

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