Table 3 Presentation of a patient with partial anomalous pulmonary venous connections and their natural history
First Author and YOP | Gender and Age | Presentation and History | Diagnostic and therapeutic interventions | |
|---|---|---|---|---|
1 | Tarun Kumar. et al. (2014) [20] | 18-year-old female | CC: Effort intolerance and easy fatigability NYHA FC: II PMH: None. PFH: None. LAB: Increased urea (48), Creatinine (2) Abdominal US: grade I renal parenchymal disease ECG: ‘P’ + right axis deviation TTE: dilated RA & RV + mild to moderate TR + TR jet gradient of 42 mm of Hg TEE: Obstructed PAPVC connecting to the IVC without any ASD or VSD Chest CT: W/O contrast due to renal dysfunction showed no pulmonary abnormality | DX: PAPVC + PHT TX: Sildenafil & low-dose diuretic to decline PHT and planned surgical anomaly correction. However, the patient did not follow up |
2 | Iqra Qamar. et al. (2018) [1] | 58-year-old-female | CC: central chest pain PMH: NSTEMI PH/EX: MRA: Revealed PAPVC, left UPV drained into the brachiocephalic vein◊ No substantial LTRS (Qp/Qs < 1.5). Delayed hyper-enhancement of the septal regions indicated an MI despite unremarkable LV and LR. | TX: Despite being asymptomatic, this patient was followed up with informed consent, and if symptoms developed, she would be considered for PAPVC surgery. |
3 | Hiroki Wakamatsu. et al. [3] | 42-year-male | CC: progressive exertional dyspnea PH/EX: An accentuated 2nd heart sound and no heart murmur were audible. NYHA FC: III CXR: Bilateral pulmonary arteries dilatation ECG: RAD and RVH and complete RBBB TTE: RVE & IVS flattening CECT: right upper and middle PVs drained into the SVC, and Pas and RV were dilated RHC: (Qp/Qs) 1.4, PAP:91 mmHg, PCWP: 12, | DX: Increased PAP due to PAPVC TX: PAH-specific drugs. Macitentan (20 mg/day) and tadalafill (10 mg/day) were immediately administered. In addition, selexipag (increasing from 2 to 10 mg/day at our outpatient clinic) Progress: The patient improved, but after a while, due to nonadherence, the surgery was successfully performed. |
4 | Pratap Upadhya et al. (2024) [21]. | 41-year-old Female | CC: exertional dyspnea, mMRC FC II, and dry cough for 3 years PH/EX: increased JVP HRCT: Non-fibrotic HP TTE: RA and RV dilatation, Mod TR with an RVSP of 48 mmHg + RAP, and a nor-mal LVEF, PCTA: NO PE but incidentally found left superior pulmonary vein draining into the left brachiocephalic vein | DX: PAPVC Treatment: Ambrisentan and tadalafil were used for 3 months, and the condition improved. |
5 | Aprateem Mukherjee et al. 2024 [4] | 27-year-old female | CC: dyspnea on exertion was TTE: ostium secundum ASD and PAPVC with RIPV draining into the coronary sinus. The pulmonary vein was seen draining into the left brachiocephalic vein. The RSVP drains normally into the LA. | DX; Double drainage PAPVC TX: Surgical treatment |
6 | Yuuki Matsui. et al. 2024 [22] | 74-year-old female | CC: Asymptomatic before chest trauma, Incidental finding of a 24 mm tumor in the right lower lobe + PAPVC CECT: PAPVC: right upper lobe pulmonary vein into the SVC Intraoperative FNA: (Qp/Qs) of 0.98 | DX; papillary adenocarcinoma with a maximum diameter of 2.1 × 1.5 cm and invasive diameter of 1.5 cm, at pathological stage IA2 with T1bN0M0 PLAN: Successful lobectomy + Not manipulating PAPVC + Good condition at 1 year FU |
7 | Gengxu He. et al. 2024 [23] | 59-year-old male | CC: short of breathing and palpitation for 3 months PH/EX: A mid-systolic 2/6 murmur over the precordium in the mitral area. TTE: RVE and PAP: 51mmHg + Mild TR CTPA: Dilation of PA + RA + RV LSPV connected to the LBCV lateral to the aortic arch, right PVs, and LIPV were seen draining normally into the LA | DX: PAPVC TX: Anastomosis of left superior vein and LA appendage through video-assisted thoracoscopy without cardiopulmonary bypass. |